Hepatosplenic T cell lymphoma: Difference between revisions

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Latest revision as of 14:42, 29 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [3]

Synonyms and Keywords: Hepatosplenic γδ T-cell lymphoma, Hepatosplenic gamma delta T-cell lymphoma, HSTCL

Overview

Hepatosplenic T cell lymphoma is a rare type of non Hodgkins lymphoma that occurs in states of immunosupression such as post organ transplant and treatment of inflammatory bowel disease. Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990. It is also known as "gamma-delta" hepatosplenic T-cell lymphoma. The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow. The incidence of hepatosplenic T cell lymphoma is 0.3 per 100000 individuals per year. If left untreated, patients can develop liver failure, pancytopenia or spleen rupture. Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic T-cell lymphoma. Chemotherapy including CHOP is the mainstay of the treatment along with bone marrow transplant and radiation therapy.

Historical Perspective

Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990.

Classification

There is no established system for the classification of hepatosplenic T cell lymphoma.

Pathophysiology

Hepatosplenic T-cell lymphoma is a peripheral T-cell lymphoma, a type of non Hodgkin's lymphoma.^[1]
It is also known as "gamma-delta hepatosplenic T-cell lymphoma".^[2]
It usually occurs in young men with history of immunosuppression including solid organ transplantation.^[3]
Patients with inflammatory bowel disease receiving immunosuppressants and anti-tumor necrosis factor-α agent are also at risk for developing hepatosplenic T-cell lymphoma.
The T-cell receptor consists of either a gamma delta or alpha-beta entity on their cell surface which are a part of the innate immune system.^[4]^[5]
Gamma delta T cells represent the first line of defense against bacterial peptides, such as heat-shock proteins.
Gamma delta T cells are CD4 and CD8 negative, but CD56 positive which is NK cell marker.
Gamma delta cells respond to a stimulus and are responsible for lymphokine production and proliferation.
Gamma delta cells are predominantly located in the spleen, liver sinusoids and intestinal epithelium.
75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
Chronic antigen stimulation in states of immunosuppression is responsible for the development of the lymphoma.
Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic T cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
Mutations in SETD2, INO80, TET3 and STAT5B occur exclusively in hepatosplenic T cell lymphoma as compared to other T and B cell lymphoma types.
The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
Splenic involvement is characterized by diffuse involvement of the red pulp with small-to-medium-sized atypical lymphocytes.
The atypical lymphocytes are present within the cords and sinuses of the red pulp.
There occurs a complete loss of the white pulp.
The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.
The bone marrow is characterized by neoplastic cells in the sinusoids.
Bone marrow infiltration results in pancytopenia.
The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
It manifests as hepatosplenomegaly without peripheral lymphadenopathy.
Pancytopenia and abnormal liver functions are the laboratory findings.
Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.

Causes

Common causes of hepatosplenic T cell lymphoma are: ^[6]^[7]

Differentiating hepatosplenic T cell lymphoma from Other Diseases

Differential diagnosis for the lymphoma is based on the below table:

Differentiating diagnosis of Lymphoma	Symptoms					Signs			Diagnosis	Additional Findings
Differentiating diagnosis of Lymphoma	Fever	Rash	Diarrhea	Abdominal pain	Weight loss	Painful lymphadenopathy	Hepatosplenomegaly	Arthritis	Lab Findings	Additional Findings
Lymphoma	+	–	–	+	+	–	+	–	Increase ESR, increased LDH	Night sweats, constant fatigue
Brucellosis	+	+	–	+	+	+	+	+	Relative lymphocytosis	Night sweats, often with characteristic smell, likened to wet hay
Typhoid fever	+	+	–	+	–	–	+	+	Decreased hemoglobin	Incremental increase in temperature initially and than sustained fever as high as 40°C (104°F)
Malaria	+	–	+	+	–	–	+	+	Microcytosis, elevated LDH	"Tertian" fever: paroxysms occur every second day
Tuberculosis	+	+	–	+	+	+	–	+	Mild normocytic anemia, hyponatremia, and hypercalcemia	Night sweats, constant fatigue
Mumps	+	–	–	–	–	+	–	–	Relative lymphocytosis, serum amylaseelevated	Parotidswelling/tenderness
Rheumatoid arthritis	–	+	–	–	–	–	–	+	ESR and CRP elevated, positive rheumatoid factor	Morning stiffness
SLE	–	+	–	+	+	–	–	+	ESR and CRP elevated, positive ANA	Fatigue
HIV	–	–	–	+	+	+	–	+	Leukopenia	Constant fatigue

Differentiating different types of T-cell Non-Hodgkin lymphoma. The gold standard for differentiation different types of Non-Hodgkin lymphoma is biopsy.

Disease		Etiology	Clinical manifestations						Paraclinical findings		Associated findings
			Clinical manifestations						Lab findings
			Symptoms				Signs		Immunochemistry	Histopathology
			Constitutional symptoms	Rash	Abdominal pain	Diarrhea	Mass	Other	Immunochemistry	Histopathology
Hodgkin's Lymphoma ^[8]^[9]^[10]^[11]^[12]		Activation of (NF-kB) Mutations in JAK-STAT pathway NF-kB leads to activation of many genes like: (ICAM-1) gene GM-CSF gene IL-6 gene IKBA gene Gain of function mutation of the MAP3K14 gene	Fever (Pel-Ebstein fever): persistent temperature >38°C (>100.4°F) Drenching night sweats Unexplained weight loss >10% of total body weight within the past 6 months	-	±	-	Painless lymphadenopathy with a rubbery consistency. Mostly above the diaphram	Pruritus Pain in lymph nodes after drinking alcohol Skin blushing or flushing Bone pain	Nodular lymphocyte predominant CD19+ CD20+ CD22+ CD15- CD30 Classical Hodgkin's lymphoma CD15+ CD30+ CD20- CD45-	Effacement of the lymph node architecture by Reed-Sternberg cells . Reed-Sternberg cell is a large often bi-nucleated cells with prominent nucleoli and an unusual immuno phenotype.	EBV infection
Non-Hodgkin's Lymphoma (B Cell Lymphoma)
Diffuse large B cell lymphoma ^[13]^[14]^[15]^[16]^[17]^[18]		Mature B-cell Lymphoma 8q24/MYC translocations Hypermutated immunoglobulin genes Mutation of BCL6, MYC, PAX5, PIM1, RhoH /TTFn, TP53 genes Translocations involving c-MYC, BCL6, and IgH gene.	Fever Night sweats Weight loss	-	+	+	Painless rapidly enlarging lymph nodes	Extranodal involvement of GI tract, lungs, and CNS	CD20 CD3 CD5 CD45 CD10 BCL-2 BCl-6 MYC IRF-4/ MUM-1 Ki-67	Centroblastic Medium-to-large-sized lymphocytes Monomorphic Immunoblastic > 90% immunoblasts Trapezoid shaped large lymphoid cells with significant basophilic cytoplasm Anaplastic Very large cells with a round, oval, or polygonal shape that may resemble Reed-Sternberg cells	_
Mantle cell lymphoma ^[19]^[20]^[21]^[22]^[23]		Mature B-cell lymphoma CD5 positive antigen in pre germinal center of B-cell Chromosomal translocation at t(11:14)	Fatigue Night sweats Weight Loss	–	+	+	Palpable masses in skin, breast, and salivary glands Generalized lymphadenopathy	Extranodal involvement of GI tract, lungs, and CNS Mental retardation	CD5⁺ B-cell antigen positive Overexpression of Cyclin D1	Germinal centers filled by small-to-medium atypical lymphocytes Nodular appearance	Abdominal distention
B-lymphoblastic leukemia/lymphoma ^[24]^[25]^[26]		Precursor B-cell lymphoma Unknown Inherited mutations of PAX5, ETV6, and TP53	+	–	–	–	Painless mass at extranodal sites mainly of the skin, bone and lymph node.	Bleeding Dyspnea Joint pain	CD20+ CD10+	Consists of small to medium-sized lymphoid cells with blastic nuclear chromatin and a high mitotic rate.	Down syndrome
Follicular lymphoma ^[27]^[28]^[29]^[30]^[31]		Mature B-cell lymphoma Reciprocal translocation t(14;18) Overexpression of BCL2	20% of patients present with: Fever Weight loss Night sweats	+	+	±	Painless peripheral adenopathy in the cervical, axillary, inguinal, and/or femoral regions Asymptomatic large abdominal mass	Seizures Chest pain Bone pain Cough Dyspnea	CD21 CD23	Nodular growth pattern	Most common clinically indolent NHL Peripheral nerve compression
Burkitt's lymphoma ^[32]^[33]^[34]^[35]^[36]^[37]^[38]^[39]		Mature B-cell lymphoma Translocation of chromosome 8 myc locus such as: t(8;14) t(2;8) t(8;22)	Fever Night sweats Unexplained weight loss	–	+	–	Swollen lymph nodes in the neck, axilla, or groin Jaw mass Abdominal masses	Ascites Proptosis	CD19 CD20 CD22 CD10 BCL6 BCL2 and TdT	Round and small nucleoli basophilic cytoplasm "Starry-sky pattern"	EBV infection HIV infection Immunosuppression Abdominal distention
B cell chronic lymphocytic leukemia/small lymphocytic lymphoma ^[40]		Mature B-cell Lymphoma Ch 13 abnormalties	33% of patients present with: Fever Weight loss Night sweats	–	–	–	Painless lymphadenopathy	May be asymptomatic Fatigue Recurrent infections Hepatosplenomegaly	CD5 CD38	Monoclonal small well differentiated B cells. Significant number of smudge cells or basket cells.	Autoimmune hemolytic anemia Red cell aplasia Autoimmune thrombocytopenia
Marginal zone lymphoma	Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type ^[41]^[42]^[43]^[44]^[45]^[46]^[47]^[48]^[49]^[50]	Mature B-cell lymphoma Recurrent translocation of such as: t(1;14)(p22;q32) t(11;18)(q21;q21) t(14;18)(q32;q21) t(3;14)(p14.1;q32)	B symptoms may or may not be present	±	+	+	Painless or painfull lymphadenopathy which can be gastric or non-gastric. Lump in the superior lateral quadrant of the orbit, breast, neck, or salivary gland	Symptoms depend on the location of the tumor: Fatigue Heartburn Fever Cough with blood in sputum, Shortness of breath	B-cell associated antigens that co-express BCL-2 CD19 CD20 CD22 CD79a Negative for: CD5 CD10 CD43 cyclin D1.	Presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria Prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features.	H. pylori infection Hashimoto's thyroiditis Sjogren’s syndrome Celiac disease.
	Splenic marginal zone lymphoma ^[51]^[52]^[53]^[54]^[55]^[56]^[57]	Mature B-cell lymphoma Clonal rearrangements of the immunoglobulin genes (heavy and light chains) Deletion 7q21-32 Translocations of the CDK6 gene located on 7q21	Fever Weight loss Night sweats	+	+	–	Painless swelling in the neck, axilla, groin, thorax, and abdomen	Chest pain Bone pain	CD20 CD79a	Small lymphocytes Transformed blasts Epithelial histocytes Plasmacytic differentiation of neoplastic cells	Hepatitis C infection
	Nodal marginal zone B-cell lymphoma ^[58]^[59]	Mature B-cell Lymphoma Stimulation of antigen receptor by autoantigen and co-stimulatory molecule CD40 Mutations in KMT2D, PTTPRD, NOTCH2, KLF2	Fever Weight loss Night sweats	+	–	–	Painless swelling in the neck, axilla, groin, thorax, and abdomen Generalized and local lymphadenopathy	Hemorrhage Dyspepsia	Follicular cells in reactive zone Centrocyte like cells in marginal zone lymphoma	Centrocytes Centroblasts Plasma cells Immunoblasts	Hepatitis C infection Chronic infectious conditions or autoimmune processes, such as: H pylori gastritis Hashimoto thyroiditis Sjögren syndrome
Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia) ^[60]^[61]^[62]^[63]^[64]		Mature B-cell Lymphoma Mutation of the MYD88 gene (L265P) Nonsense or frameshift mutations in the CXR4 gene	Weakness Anorexia Weight loss Fever	–	–	+	Generalized and local lymphadenopathy	Retinal hemorrhage with blurring of vision Vertigo Dizziness Headache Nystagmus Tinnitus Ataxia Bleeding	Express pan B-cell antigens CD19 CD20 CD22 CD79A CD5 Variable expression of CD11c CD43, CD25 Majority express: IgM surface immunoglobulin Fewer express IgG or IgA Lack IgD	Russell bodies (intracytoplasmic inclusions) and Dutcher bodies (intranuclear inclusions) Prominent epithelioid histiocytes	Diffuse large B-cell lymphoma Myelodysplastic syndrome/Acute myeloid leukemia Brain tumor Renal MALT lymphoma
Hairy cell leukemia ^[65]^[66]^[67]^[68]^[69]		Production of cytokines, such as TNF alpha and IL-2R, provide important stimuli for malignant B cells proliferation in hairy cell leukemia. The p38-MAPK-JNK cascade The MEK-ERK cascade The Phosphatidylinositol 3 kinase (PI3K)-AKT cascade	Fever Night sweats Fatigue Easy bruising or bleeding Generalized weakness Weight loss Recurrent infections Early satiety	–	+	–	Pallor Petechiae Splenomegaly	N/A	Annexin A1 CD20 CD25 CD103 CD19 CD11c FMC7	Small cells with "fried egg"-like appearance Well-demarcated thread-like cytoplasmic extensions Clear cytoplasm Central round nucleus Peri-nuclear clearing ("water-clear rim" appearance)	Abdominal distention
Disease		Etiology	Constitutional symptoms	Rash	Abdominal pain	Diarrhea	Mass/Lesion	Other	Immunochemistry	Histopathology	Associated findings
Non-Hodgkin's Lymphoma (T Cell Lymphoma)
Precursor T-cell lymphoblastic leukemia/lymphoma ^[70]^[71]		Precursor T-cell lymphomas	Fever Fatigue Weight loss Night sweats	+	-	-	In most patients the mediastinal mass is anterior; associated with pleural effusions Can produce such complications as superior vena cava syndrome Skin and bone abnormalities Enlarged liver and/or spleen Tracheal obstruction Pericardial effusions	Skin rash Chest pain	CD3 CD7 TDT	Blast-like cells with scant cytoplasm, convoluted nuclei, fine chromatin, indistinct nucleoli. Some lymphoblasts have cytoplasmic pseudopods	Males in their teens to early twenties Lymphadenopathy in cervical, supraclavicular, axillary ,and mediastinum
T-cell granular lymphocytic ^[72]^[73]^[74]		Clonal proliferation of CD8 T cells Constant immune stimulation Absence of homeostatic apoptosis	Fatigue Fever Night sweats	-	-	-	Lymphadenopathy Hepatosplenomegaly	Frequent infections	CD8 CD57 TCRαβ CD3 CD16 Granzyme b TIA1	Medium / large lymphocytes with abundant cytoplasm having azurophilic granules	May be associated with the following conditions: Monoclonal gammopathy of unknown significance (MGUS) B-chronic lymphocytic leukemia (B-CLL) Hairy cell leukemia Myeloid hypoplasia
T-cell prolymphocytic leukemia ^[75]		Abnormal proliferation of mature T cells (post thymic)	Fever Fatigue Weight loss Night sweats	+	-	-	Lymphadenopathy Splenomegaly Hepatomegaly	Skin rash	CD2 CD3 CD4 CD5 CD7 CD56 TCL1	Lymphocytes with abundant basophilic, nongranular cytoplasm, atypical nucleus Cytoplasmic protrusions (blebs)	Pleural effusion
Adult T cell leukemia/lymphoma ^[76]^[77]^[78]		Mature T-cell lymphoma Human T-lymphotrophic virus 1 infection	Hepatosplenomagaly T-cell deficiency leading immunodeficiency causing opportunistic infection	+	-	-	Hepatosplenomegaly	Hypercalcemia Skin lesions Lytic bone lesions Elevated LDH	Adult T-cell leukemia cells are typically positive for: CD2 CD4 CD5 CD8	Charecterstic leukemia cells with sharp nuclear indentations and a prominent nucleoli "Cloverleaf" or "flower" cells	Hypercalcemia
Anaplastic large cell lymphoma ^[79]^[80]^[81]^[82]		Mature T-cell lymphoma	Fever Weight loss Night sweats	+	+	-	Painless swelling in : Neck Axilla Groin Thorax Abdomen	Skin rash Chest pain Abdominal pain Bone pain	Strongly immunoreactive for : CD30 Anaplastic lymphoma kinase (ALK) EMA vimentin	Nucleoli tend to be more prominent The cytoplasm may be either basophilic or eosinophilic and the cell might have many nuclei with dispersed or clumped chromatin	May be caused by textured breast implants
Cutaneous T-cell lymphoma	Mycosis fungoides / Sézary syndrome ^[83]^[84]^[85]	Mature T-cell Lymphoma The tumor cells originate from memory T cells or skin homing CD4+ T cells expressing cutaneous lymphocyte antigen (CLA) and chemokine receptors CCR4and CCR7. It is understood that cutaneous T cell lymphoma (maycosis fungoides, Sezary sydrome ) is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.	Weight loss Malaise and fatigue Anemia	+	+	-	Cutaneous manifestations	Lymphadenopathy May progress to Sezary syndrome	Beta F1+ CD2-/+ CD3+ CD3- (CD4-positive variant) CD4+ (CD4-positive variant) CD4- CD5- CD7+/- CD8+ CD8- (CD4-positive variant), Granzyme B+ perforin+	Polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells These cells may line up individually along the epidermal basal layer The presence of spongiosis is highly suggestive of mycosis fungoides	Epidermal atrophy or poikiloderma Generalized itching(pruritus) Pain in the affected area of the skin. Insomnia Red (erythematous) patches scattered over the skin of the trunk and the extremities
Peripheral T-cell lymphoma	Subcutaneous panniculitis-like T-cell lymphoma ^[86]^[87]^[88]	Mature T-cell lymphoma	Fever Chills Weight loss Night sweats	-	-	-	Painless swellings on: Extremities Trunks	Myalgias	Positive for: BetaF1 CD8 CD30 CLA T-cell intracellular antigen (T1A1) Perforin	Atypical lymphoid cells Fat necrosis Karyorrhexis	Subcutaneous panniculitis-like T-cell lymphoma can mimic the following conditions making it difficult to diagnose Begnign panniculitis Eczema Cellulitis Dermatitis
	Hepatosplenic T-cell lymphoma ^[89]^[90]^[91]	Mature T-cell Lymphoma Inflammatory bowel disease Organ transplant patients (reciever) Immunosuppresent medications Thiopurines Infliximab Cyclophosphamide Vincristine Doxorubicin	Fever Fatigue Weight loss Night sweats	+	+	-	Painless swelling in : Neck Axilla Groin Thorax Abdomen	Skin rash Chest pain Abdominal pain Bone pain	CD2 CD3 CD7 CD16 CD56 or CD57 TIA1 Granzyme m Fas ligand	Intermediate sized tumor cells with clear cytoplasm, oval nuclei, slightly dispersed condensed chromatin, inconspicuous nucleoli	Patients with inflammatory bowel diseases taking immunosuppressant and anti-tumor necrosis factor-α agent are also have a higher risk for developing hepatosplenic T-cell lymphoma
	Enteropathy-type intestinal T-cell lymphoma ^[92]^[93]^[94]	Mature T-cell lymphoma	Fever Weight loss Night sweats Diarrhea	+	+	+	Painless swelling in the : Neck axilla groin thorax, abdomen	Skin rash Chest pain Abdominal pain Bone pain	CD3+ CD4- CD5- CD7+ CD30+ CD56+ CD103+ CD8+/- TCR beta+/-	Lymphomatous infiltrate with neoplastic large cells May have pleomorphic, multinucleated cells Adjacent mucosa shows villous atrophy, crypt hyperplasia, increased inflammatory cells Lymphocytosis	Mostly diagnosed in the small intestine
	Extranodal T-cell lymphoma, nasal type ^[95]^[96]^[97]	Mature T-cell lymphoma	Fever Weight loss Night sweats	+	-	-	Painless swelling in : Neck Axilla Groin Thorax Abdomen	Skin rash Chest pain Abdominal pain Bone pain	Cytoplasmic CD3 epsilon Granzyme B Perforin CD2 CD56	Medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells Lymphoma cells may be admixed with a polymorphic infiltrate of non-neoplastic inflammatory cells	Protrusion of eye Swelling of the face Discharge from the nose Nose bleeds Blockage of the nasal passages
	Angioimmunoblastic T-cell lymphoma ^[98]^[99]^[100]	Mature T-cell lymphoma	Fever Weight loss Night sweats Edema Fatigue	+	+	-	Painless swelling in : Neck Axilla Groin Thorax Abdomen	Skin rash abdominal pain bone pain dark urine shortness of breath chronic pain and swelling of joints Chest pain	CD2 CD3 CD4 CD5 CD10 CD20 Focal positivity for CXCL13	Leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes Hyperplastic germinal centers and Reed-Sternberg cells	Monoclonal gammopathy Autoimmune hemolytic anemia
	Peripheral T-cell lymphoma, unspecified ^[101]^[102]	Mature T-cell lymphoma	Fatigue Fever Frequent infections	-	+	-	Lymphadenopathy Hepatosplenomegaly	Lymphadenopathy (swollen ) Thirst Nausea Vomiting Skin and bone abnormalities Enlarged liver and/or spleen	CD2 CD3 TCR β F1 Variable CD4 CD5 CD7 Occasional CD56 Cytotoxic granule expression	Clear cytoplasm, resemble Reed-Sternberg cells, with irregular, pleomorphic, hyperchromatic or nuclei A lot of mitotic figures; very broad cytologic spectrum	Endogenous immunosupression Patients may present with p.jiroveci or varicella zoster infection Can be confused with other infectious or rheumatologic diseases

Epidemiology and Demographics

The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year.
It occurs in younger group of patients, most cases falling in 20-40 years of age group.
Men are more affected than the females.

Risk Factors

Common risk factors include:

Autoimmune diseases
Patients on immunosuppresant medications.
Patients on chemotherapy.
Inflammatory bowel disease
Organ transplant patients.

Screening

There is insufficient evidence to recommend routine screening for hepatosplenic T cell lymphoma.

Natural History, Complications, and Prognosis

Natural history

Patients have a history of immunosupression such as inflammatory bowel disease under treatment or organ transplant.^[103]
The mean age group is 35 years and most of the patients are males.
Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no lymphadenopathy.
Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
If left untreated, patients can develop liver failure, pancytopenia, or spleen rupture.

Complications

Hepatomegaly
Hepatic failure
Portal vein thrombosis
Splenomegaly
Splenic infarction
Spleen rupture
Splenic vein thrombosis
Anemia
Thrombocytopenia
Neutropenia
Neurological dysfunction if metastasizes to brain.
Intestinal perforation
Intestinal obstruction

Prognosis

The prognosis is very poor, with patients dying within 2-3 years of diagnosis even after recieving treatment^[104].

Diagnosis

Diagnostic Study of Choice

Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic T cell lymphoma^[105].
CT scan and PET scan are used to assess the spread of the lymphoma.

Symptoms

The most common symtoms are^[106]:

Fever
Weight loss
Night sweats
Pain abdomen
Jaundice
Fatigue
Recurrent infections
Bleeding

Physical Examination

Temperature

Fever is often present

Skin

Rash

Thorax

Pleural effusion
Chest tenderness

Abdomen

Splenomegaly
Hepatomegaly
Ascites
Abdomen tenderness^[103]

Extremities

Bone tenderness^[103]

Laboratory Findings

Biopsy of the tumor:
- Histology - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
- Flow cytometry and immunophenotyping - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression^[107].
- Karyotyping - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome^[108].
Bone marrow biopsy; The bone marrow is characterized by neoplastic cells in the sinusoids.
Complete blood count; Pancytopenia
Liver function tests: Deranged LFT
- Elevated transaminases
- Hyperbilirubinemia
- Hypoproteinemia
- Elevated alkaline phosphatase
- Elevated PT/INR

Electrocardiogram

There are no ECG findings associated with hepatosplenic T cell lymphoma.

X-ray

There are no x-ray findings associated with hepatosplenic T cell lymphoma but pleural effusion might be present.

Echocardiography or Ultrasound

There are no echocardiography findings associated with hepatosplenic T cell lymphoma.
On ultrasound of abdomen:
- Hepatomegaly
- Splenomegaly

CT scan

Tumor mass can be seen in liver or spleen or both.

MRI

Tumor mass can be seen in liver or spleen or both.

Other Imaging Findings

PET scan: On PET scan, tumor mass can be seen in liver or spleen or if it has metastasized to any other organ.

Treatment

Medical Therapy

Induction therapy^[109]
Consolidation therapy
- Radiation therapy
- Allogenic bone marrow transplant^[110]
- Autologous bone marrow transplant
Additional medications given during chemotherapy:

Surgery

Surgical intervention is not recommended for the management of hepaosplenic T cell lymphoma.

Primary Prevention

There are no established measures for the primary prevention of hepatosplenic T cell lymphoma.

Secondary Prevention

There are no established measures for the secondary prevention of hepatosplenic T cell lymphoma.

References

↑ Armitage JO (2017). "The aggressive peripheral T-cell lymphomas: 2017". Am J Hematol. 92 (7): 706–715. doi:10.1002/ajh.24791. PMID 28516671.
↑ Brandt PH, Rahmat LT, Ali SS (2019). "A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis". Clin Case Rep. 7 (1): 164–169. doi:10.1002/ccr3.1924. PMC 6333078. PMID 30656034.
↑ Choi Y, Jeon SY, Yoo WH (2018). "Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis". J Clin Rheumatol. doi:10.1097/RHU.0000000000000805. PMID 29933321.
↑ Gowda L, Foss F (2019). "Hepatosplenic T-Cell Lymphomas". Cancer Treat Res. 176: 185–193. doi:10.1007/978-3-319-99716-2_9. PMID 30596219.
↑ Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M; et al. (1990). "Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas". Am J Pathol. 137 (3): 617–28. PMC 1877506. PMID 1698028.
↑ Yabe M, Miranda RN, Medeiros LJ (2018). "Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors". Hum Pathol. 74: 5–16. doi:10.1016/j.humpath.2018.01.005. PMID 29337025.
↑ Gallamini A, Stelitano C, Calvi R, Bellei M, Mattei D, Vitolo U; et al. (2004). "Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study". Blood. 103 (7): 2474–9. doi:10.1182/blood-2003-09-3080. PMID 14645001.
↑ Mauch PM, Kalish LA, Kadin M, Coleman CN, Osteen R, Hellman S (March 1993). "Patterns of presentation of Hodgkin disease. Implications for etiology and pathogenesis". Cancer. 71 (6): 2062–71. PMID 8443755.
↑ Gobbi PG, Cavalli C, Gendarini A, Crema A, Ricevuti G, Federico M, Di Prisco U, Ascari E (December 1985). "Reevaluation of prognostic significance of symptoms in Hodgkin's disease". Cancer. 56 (12): 2874–80. PMID 4052959.
↑ Buri C, Körner M, Schärli P, Cefai D, Uguccioni M, Mueller C, Laissue JA, Mazzucchelli L (March 2001). "CC chemokines and the receptors CCR3 and CCR5 are differentially expressed in the nonneoplastic leukocytic infiltrates of Hodgkin disease". Blood. 97 (6): 1543–8. PMID 11238088.
↑ Peh SC, Looi LM, Pallesen G (March 1997). "Epstein-Barr virus (EBV) and Hodgkin's disease in a multi-ethnic population in Malaysia". Histopathology. 30 (3): 227–33. PMID 9088951.
↑ Andriko JA, Aguilera NS, Nandedkar MA, Abbondanzo SL (April 1997). "Childhood Hodgkin's disease in the United States: an analysis of histologic subtypes and association with Epstein-Barr virus". Mod. Pathol. 10 (4): 366–71. PMID 9110300.
↑ Colomo, L.; López-Guillermo, A; Perales, M; Rives, S; Martínez, A; Bosch, F; Colomer, D; Falini, B; Montserrat, E; Campo, E (2002). "Clinical impact of the differentiation profile assessed by immunophenotyping in patients with diffuse large B-cell lymphoma". Blood. 101 (1): 78–84. doi:10.1182/blood-2002-04-1286. PMID 12393466.
↑ Hans, C. P.; Weisenburger, D. D.; Greiner, T. C.; Gascoyne, R. D.; Delabie, J; Ott, G; Müller-Hermelink, H. K.; Campo, E; Braziel, R. M.; Jaffe, E. S.; Pan, Z; Farinha, P; Smith, L. M.; Falini, B; Banham, A. H.; Rosenwald, A; Staudt, L. M.; Connors, J. M.; Armitage, J. O.; Chan, W. C. (2004). "Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray". Blood. 103 (1): 275–82. doi:10.1182/blood-2003-05-1545. PMID 14504078.
↑ Muris, JJF; Meijer, Cjlm; Vos, W; Van Krieken, Jhjm; Jiwa, NM; Ossenkoppele, GJ; Oudejans, JJ (2006). "Immunohistochemical profiling based on Bcl-2, CD10 and MUM1 expression improves risk stratification in patients with primary nodal diffuse large B cell lymphoma". The Journal of Pathology. 208 (5): 714–23. doi:10.1002/path.1924. PMID 16400625.
↑ Korkolopoulou P, Vassilakopoulos T, Milionis V, Ioannou M (2016). "Recent Advances in Aggressive Large B-cell Lymphomas: A Comprehensive Review". Adv Anat Pathol. 23 (4): 202–43. doi:10.1097/PAP.0000000000000117. PMID 27271843.
↑ Swerdlow SH, et al (editors). WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edition. 2008. International Agency for Research on Cancer (IARC), Lyon, Franc
↑ Tilly H, et al. Diffuse large B-cell lymphoma (DLBCL): ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology. 2015; 26: v116-v125
↑ Itziar Salaverria, Cristina Royo, Alejandra Carvajal-Cuenca, Guillem Clot, Alba Navarro, Alejandra Valera, Joo Y. Song, Renata Woroniecka, Grzegorz Rymkiewicz, Wolfram Klapper, Elena M. Hartmann, Pierre Sujobert, Iwona Wlodarska, Judith A. Ferry, Philippe Gaulard, German Ott, Andreas Rosenwald, Armando Lopez-Guillermo, Leticia Quintanilla-Martinez, Nancy L. Harris, Elaine S. Jaffe, Reiner Siebert, Elias Campo & Silvia Bea (2013). "CCND2 rearrangements are the most frequent genetic events in cyclin D1(-) mantle cell lymphoma". Blood. 121 (8): 1394–1402. doi:10.1182/blood-2012-08-452284. PMID 23255553. Unknown parameter |month= ignored (help)
↑ Markus Tiemann, Carsten Schrader, Wolfram Klapper, Martin H. Dreyling, Elias Campo, Andrew Norton, Francoise Berger, Philip Kluin, German Ott, Stephano Pileri, Ennio Pedrinis, Alfred C. Feller, Hartmut Merz, Dirk Janssen, Martin L. Hansmann, Han Krieken, Peter Moller, Harald Stein, Michael Unterhalt, Wolfgang Hiddemann & Reza Parwaresch (2005). "Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network". British journal of haematology. 131 (1): 29–38. doi:10.1111/j.1365-2141.2005.05716.x. PMID 16173960. Unknown parameter |month= ignored (help)
↑ L. H. Argatoff, J. M. Connors, R. J. Klasa, D. E. Horsman & R. D. Gascoyne (1997). "Mantle cell lymphoma: a clinicopathologic study of 80 cases". Blood. 89 (6): 2067–2078. PMID 9058729. Unknown parameter |month= ignored (help)
↑ Markus Tiemann, Carsten Schrader, Wolfram Klapper, Martin H. Dreyling, Elias Campo, Andrew Norton, Francoise Berger, Philip Kluin, German Ott, Stephano Pileri, Ennio Pedrinis, Alfred C. Feller, Hartmut Merz, Dirk Janssen, Martin L. Hansmann, Han Krieken, Peter Moller, Harald Stein, Michael Unterhalt, Wolfgang Hiddemann & Reza Parwaresch (2005). "Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network". British journal of haematology. 131 (1): 29–38. doi:10.1111/j.1365-2141.2005.05716.x. PMID 16173960. Unknown parameter |month= ignored (help)
↑ Julie M. Vose (2017). "Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management". American journal of hematology. 92 (8): 806–813. doi:10.1002/ajh.24797. PMID 28699667. Unknown parameter |month= ignored (help)
↑ Lin P, Jones D, Dorfman DM, Medeiros LJ (November 2000). "Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement". Am. J. Surg. Pathol. 24 (11): 1480–90. PMID 11075849.
↑ Javed Z, Hanif F (August 2018). "A Rare Presentation of Precursor B-cell Lymphoblastic Lymphoma in a Child". Cureus. 10 (8): e3238. doi:10.7759/cureus.3238. PMC 6209516. PMID 30410844.
↑ Kim JY, Om SY, Shin SJ, Kim JE, Yoon DH, Suh C (December 2014). "Case series of precursor B-cell lymphoblastic lymphoma". Blood Res. 49 (4): 270–4. doi:10.5045/br.2014.49.4.270. PMC 4278010. PMID 25548762.
↑ Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES (2014). "Early lymphoid lesions: conceptual, diagnostic and clinical challenges". Haematologica. 99 (9): 1421–32. doi:10.3324/haematol.2014.107938. PMC 4562530. PMID 25176983.
↑ Lorsbach RB, Shay-Seymore D, Moore J, Banks PM, Hasserjian RP, Sandlund JT; et al. (2002). "Clinicopathologic analysis of follicular lymphoma occurring in children". Blood. 99 (6): 1959–64. PMID 11877266.
↑ Overview at UMDNJ
↑ Bosga-Bouwer AG, Haralambieva E, Booman M; et al. (November 2005). "BCL6 alternative translocation breakpoint cluster region associated with follicular lymphoma grade 3B". Genes Chromosomes Cancer. 44 (3): 301–4. doi:10.1002/gcc.20246. PMID 16075463.
↑ Winberg CD, Nathwani BN, Bearman RM, Rappaport H (1981). "Follicular (nodular) lymphoma during the first two decades of life: a clinicopathologic study of 12 patients". Cancer. 48 (10): 2223–35. PMID 7028244.
↑ Burkitt's Lymphoma. Wikibooks. https://en.wikibooks.org/wiki/Radiation_Oncology/NHL/Burkitt_lymphoma#Pathology Accessed on October,5 2015
↑ Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L (2003). "Burkitt's lymphoma: new insights into molecular pathogenesis". J. Clin. Pathol. 56 (3): 188–92. PMC 1769902. PMID 12610094. Unknown parameter |month= ignored (help)
↑ Chuang, Shih-Sung; Ye, Hongtao; Du, Ming-Qing; Lu, Chin-Li; Dogan, Ahmet; Hsieh, Pin-Pen; Huang, Wan-Ting; Jung, Yun-Chih (2007). "Histopathology and Immunohistochemistry in Distinguishing Burkitt Lymphoma From Diffuse Large B-Cell Lymphoma With Very High Proliferation Index and With or Without a Starry-Sky Pattern". American Journal of Clinical Pathology. 128 (4): 558–564. doi:10.1309/EQJR3D3V0CCQGP04. ISSN 0002-9173.
↑ Magrath IT, Simon RM (1976). "Immunosuppression in Burkitt's lymphoma. II. Peripheral blood lymphocyte populations related to clinical status". Int J Cancer. 18 (4): 399–408. PMID 977186.
↑ Basavaraj A, Shinde A, Kulkarni R, Kadam DB, Chugh A (2014). "HIV associated Burkitt's lymphoma". J Assoc Physicians India. 62 (8): 723–7. PMID 25856946.
↑ Rowe, Martin; Fitzsimmons, Leah; Bell, Andrew I (2014). "Epstein-Barr virus and Burkitt lymphoma". Chinese Journal of Cancer. doi:10.5732/cjc.014.10190. ISSN 1000-467X.
↑ Ekemen S, Uzay A, Bassullu N, Dikicioglu-Cetin E, Matsuda K, Ince U; et al. (2018). "Does it take three to tango? An unsuspected multimorbidity of CD8+ T cell lymphoproliferative disorder, malaria, and EBV infection". Malar J. 17 (1): 349. doi:10.1186/s12936-018-2497-9. PMC 6173833. PMID 30290813.
↑ Kretzmer H, Bernhart SH, Wang W, Haake A, Weniger MA, Bergmann AK; et al. (2015). "DNA methylome analysis in Burkitt and follicular lymphomas identifies differentially methylated regions linked to somatic mutation and transcriptional control". Nat Genet. 47 (11): 1316–1325. doi:10.1038/ng.3413. PMC 5444523. PMID 26437030.
↑ Klein, Ulf; Tu, Yuhai; Stolovitzky, Gustavo A.; Mattioli, Michela; Cattoretti, Giorgio; Husson, Hervé; Freedman, Arnold; Inghirami, Giorgio; Cro, Lilla; Baldini, Luca; Neri, Antonino; Califano, Andrea; Dalla-Favera, Riccardo (2001). "Gene Expression Profiling of B Cell Chronic Lymphocytic Leukemia Reveals a Homogeneous Phenotype Related to Memory B Cells". The Journal of Experimental Medicine. 194 (11): 1625–1638. doi:10.1084/jem.194.11.1625. ISSN 0022-1007.
↑ Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016
↑ Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016
↑ Kinkade, Zoe; Esan, Olukemi A.; Rosado, Flavia G.; Craig, Michael; Vos, Jeffrey A. (2015). "Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report". Diagnostic Pathology. 10 (1). doi:10.1186/s13000-015-0353-6. ISSN 1746-1596.
↑ Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016
↑ Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016
↑ Signs and symptoms of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 28, 2016
↑ Clinical presentation of orbital lymphoma. Dr Craig Hacking and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/orbital-lymphoma. Accessed on January 28, 2016
↑ Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016
↑ Taal, B G; Boot, H; van Heerde, P; de Jong, D; Hart, A A; Burgers, J M (1 October 1996). "Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept". Gut. 39 (4): 556–561. doi:10.1136/gut.39.4.556.
↑ Bacon CM, Du MQ, Dogan A (2007). "Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists". J Clin Pathol. 60 (4): 361–72. doi:10.1136/jcp.2005.031146. PMC 2001121. PMID 16950858.
↑ Hernández JM, García JL, Gutiérrez NC, Mollejo M, Martínez-Climent JA, Flores T, González MB, Piris MA, San Miguel JF (May 2001). "Novel genomic imbalances in B-cell splenic marginal zone lymphomas revealed by comparative genomic hybridization and cytogenetics". Am. J. Pathol. 158 (5): 1843–50. doi:10.1016/S0002-9440(10)64140-5. PMC 1891967. PMID 11337382.
↑ Andersen CL, Gruszka-Westwood A, Atkinson S, Matutes E, Catovsky D, Pedersen RK, Pedersen BB, Pulczynski S, Hokland P, Jacobsen E, Koch J (January 2005). "Recurrent genomic imbalances in B-cell splenic marginal-zone lymphoma revealed by comparative genomic hybridization". Cancer Genet. Cytogenet. 156 (2): 122–8. doi:10.1016/j.cancergencyto.2004.04.026. PMID 15642391.
↑ Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F (September 2010). "Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group". Blood. 116 (9): 1479–88. doi:10.1182/blood-2010-02-267476. PMID 20479288.
↑ Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on December 22, 2015
↑ Weng WK, Levy S (July 2003). "Hepatitis C virus (HCV) and lymphomagenesis". Leuk. Lymphoma. 44 (7): 1113–20. doi:10.1080/1042819031000076972. PMID 12916862.
↑ Quinn ER, Chan CH, Hadlock KG, Foung SK, Flint M, Levy S (December 2001). "The B-cell receptor of a hepatitis C virus (HCV)-associated non-Hodgkin lymphoma binds the viral E2 envelope protein, implicating HCV in lymphomagenesis". Blood. 98 (13): 3745–9. PMID 11739181.
↑ Chuang SS, Liao YL, Chang ST, Hsieh YC, Kuo SY, Lu CL, Hwang WS, Lin IH, Tsao CJ, Huang WT (July 2010). "Hepatitis C virus infection is significantly associated with malignant lymphoma in Taiwan, particularly with nodal and splenic marginal zone lymphomas". J. Clin. Pathol. 63 (7): 595–8. doi:10.1136/jcp.2010.076810. PMID 20530156.
↑ Spina, V.; Khiabanian, H.; Messina, M.; Monti, S.; Cascione, L.; Bruscaggin, A.; Spaccarotella, E.; Holmes, A. B.; Arcaini, L.; Lucioni, M.; Tabbo, F.; Zairis, S.; Diop, F.; Cerri, M.; Chiaretti, S.; Marasca, R.; Ponzoni, M.; Deaglio, S.; Ramponi, A.; Tiacci, E.; Pasqualucci, L.; Paulli, M.; Falini, B.; Inghirami, G.; Bertoni, F.; Foa, R.; Rabadan, R.; Gaidano, G.; Rossi, D. (2016). "The genetics of nodal marginal zone lymphoma". Blood. 128 (10): 1362–1373. doi:10.1182/blood-2016-02-696757. ISSN 0006-4971.
↑ Nodal marginal zone lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/nodal-marginal-zone-lymphoma/?region=nb Accessed on March 4, 2016
↑ Steven P. Treon, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Patricia Sheehy, Robert J. Manning, Christopher J. Patterson, Christina Tripsas, Luca Arcaini, Geraldine S. Pinkus, Scott J. Rodig, Aliyah R. Sohani, Nancy Lee Harris, Jason M. Laramie, Donald A. Skifter, Stephen E. Lincoln & Zachary R. Hunter (2012). "MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia". The New England journal of medicine. 367 (9): 826–833. doi:10.1056/NEJMoa1200710. PMID 22931316. Unknown parameter |month= ignored (help)
↑ Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT (2014). "Renal MALT lymphoma associated with Waldenström macroglobulinemia". J. Formos. Med. Assoc. 113 (4): 255–7. doi:10.1016/j.jfma.2011.02.007. PMID 24685302.
↑ Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT (2014). "Renal MALT lymphoma associated with Waldenström macroglobulinemia". J. Formos. Med. Assoc. 113 (4): 255–7. doi:10.1016/j.jfma.2011.02.007. PMID 24685302.
↑ García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A; et al. (2001). "Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases". Br J Haematol. 115 (3): 575–82. PMID 11736938.
↑ Merlini G, Baldini L, Broglia C, Comelli M, Goldaniga M, Palladini G; et al. (2003). "Prognostic factors in symptomatic Waldenstrom's macroglobulinemia". Semin Oncol. 30 (2): 211–5. doi:10.1053/sonc.2003.50064. PMID 12720138.
↑ Matutes E (October 2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol. Oncol. Clin. North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.
↑ Matutes E (October 2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol. Oncol. Clin. North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.
↑ Xi L, Arons E, Navarro W, Calvo KR, Stetler-Stevenson M, Raffeld M, Kreitman RJ (April 2012). "Both variant and IGHV4-34-expressing hairy cell leukemia lack the BRAF V600E mutation". Blood. 119 (14): 3330–2. doi:10.1182/blood-2011-09-379339. PMC 3321859. PMID 22210875.
↑ Boyd EM, Bench AJ, van 't Veer MB, Wright P, Bloxham DM, Follows GA, Scott MA (December 2011). "High resolution melting analysis for detection of BRAF exon 15 mutations in hairy cell leukaemia and other lymphoid malignancies". Br. J. Haematol. 155 (5): 609–12. doi:10.1111/j.1365-2141.2011.08868.x. PMID 21910720.
↑ Forconi F, Raspadori D, Lenoci M, Lauria F (February 2005). "Absence of surface CD27 distinguishes hairy cell leukemia from other leukemic B-cell malignancies". Haematologica. 90 (2): 266–8. PMID 15710587.
↑ Shelly D, Gujral S (2017). "Early T-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma: Immunohistochemical Evaluation of Four Lymph Node Biopsies". J Clin Diagn Res. 11 (7): EL01–EL02. doi:10.7860/JCDR/2017/29352.10164. PMC 5583929. PMID 28892922.
↑ You MJ, Medeiros LJ, Hsi ED (2015). "T-lymphoblastic leukemia/lymphoma". Am J Clin Pathol. 144 (3): 411–22. doi:10.1309/AJCPMF03LVSBLHPJ. PMID 26276771.
↑ Rose, M. G. (2004). "T-Cell Large Granular Lymphocyte Leukemia and Related Disorders". The Oncologist. 9 (3): 247–258. doi:10.1634/theoncologist.9-3-247. ISSN 1083-7159.
↑ Liu EB, Chen HS, Zhang PH, Li ZQ, Sun Q, Yang QY; et al. (2012). "[Hematopathologic features of T-cell large granular lymphocytic leukemia]". Zhonghua Bing Li Xue Za Zhi. 41 (4): 229–33. doi:10.3760/cma.j.issn.0529-5807.2012.04.004. PMID 22800517.
↑ Osuji N, Matutes E, Catovsky D, Lampert I, Wotherspoon A (2005). "Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review". Am J Surg Pathol. 29 (7): 935–41. PMID 15958859.
↑ Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.
↑ Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
↑ Matutes E (2007) Adult T-cell leukaemia/lymphoma. J Clin Pathol 60 (12):1373-7. DOI:10.1136/jcp.2007.052456 PMID: 18042693
↑ Shimoyama M (1991). "Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87)". Br J Haematol. 79 (3): 428–37. PMID 1751370.
↑ Al-Ahmad, Selma; Maertens, Vincent; Libeer, Christophe; Schelfhout, Vera; Vanhoenacker, Filip; Boeckx, Nancy; Vandevenne, Marleen (2017). "The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature". Acta Clinica Belgica. 72 (6): 454–460. doi:10.1080/17843286.2017.1312057. ISSN 1784-3286.
↑ Yu L, Yan LL, Yang SJ (2014). "Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature". Int J Clin Exp Pathol. 7 (8): 4806–16. PMC 4152041. PMID 25197351.
↑ de Campos FPF, Zerbini MCN, Felipe-Silva A, Simões AB, Lovisolo SM, da Fonseca LG; et al. (2014). "Unusual clinical presentation of anaplastic large cell lymphoma". Autops Case Rep. 4 (1): 21–27. doi:10.4322/acr.2014.004. PMC 5470561. PMID 28652989.
↑ Watanabe M, Ogawa Y, Itoh K; et al. (January 2008). "Hypomethylation of CD30 CpG islands with aberrant JunB expression drives CD30 induction in Hodgkin lymphoma and anaplastic large cell lymphoma". Lab. Invest. 88 (1): 48–57. doi:10.1038/labinvest.3700696. PMID 17965727.
↑ Foss, Francine M.; Girardi, Michael (2017). "Mycosis Fungoides and Sezary Syndrome". Hematology/Oncology Clinics of North America. 31 (2): 297–315. doi:10.1016/j.hoc.2016.11.008. ISSN 0889-8588.
↑ Campbell, J. J.; Clark, R. A.; Watanabe, R.; Kupper, T. S. (2010). "Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors". Blood. 116 (5): 767–771. doi:10.1182/blood-2009-11-251926. ISSN 0006-4971.
↑ Vonderheid, Eric C.; Bernengo, Maria Grazia; Burg, Günter; Duvic, Madeleine; Heald, Peter; Laroche, Liliane; Olsen, Elise; Pittelkow, Mark; Russell-Jones, Robin; Takigawa, Masahiro; Willemze, Rein (2002). "Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas". Journal of the American Academy of Dermatology. 46 (1): 95–106. doi:10.1067/mjd.2002.118538. ISSN 0190-9622.
↑ Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016
↑ Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016
↑ Parveen Z, Thompson K (2009). "Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas". Arch Pathol Lab Med. 133 (2): 303–8. doi:10.1043/1543-2165-133.2.303. PMID 19195975.
↑ van de Meeberg MM, Derikx LA, Sinnige HA, Nooijen P, Schipper DL, Nissen LH (2016). "Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy". World J Gastroenterol. 22 (47): 10465–10470. doi:10.3748/wjg.v22.i47.10465. PMC 5175260. PMID 28058028.
↑ Shi, Yang; Wang, Endi (2015). "Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer–Cell Neoplasms". Archives of Pathology & Laboratory Medicine. 139 (9): 1173–1180. doi:10.5858/arpa.2014-0079-RS. ISSN 0003-9985.
↑ Alsohaibani FI, Abdulla MA, Fagih MM (2011). "Hepatosplenic T-cell lymphoma". Indian J Hematol Blood Transfus. 27 (1): 39–42. doi:10.1007/s12288-010-0051-1. PMC 3102508. PMID 22379294.
↑ Delabie, J.; Holte, H.; Vose, J. M.; Ullrich, F.; Jaffe, E. S.; Savage, K. J.; Connors, J. M.; Rimsza, L.; Harris, N. L.; Muller-Hermelink, K.; Rudiger, T.; Coiffier, B.; Gascoyne, R. D.; Berger, F.; Tobinai, K.; Au, W. Y.; Liang, R.; Montserrat, E.; Hochberg, E. P.; Pileri, S.; Federico, M.; Nathwani, B.; Armitage, J. O.; Weisenburger, D. D. (2011). "Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project". Blood. 118 (1): 148–155. doi:10.1182/blood-2011-02-335216. ISSN 0006-4971.
↑ Bautista-Quach MA, Ake CD, Chen M, Wang J (September 2012). "Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features". J Gastrointest Oncol. 3 (3): 209–25. doi:10.3978/j.issn.2078-6891.2012.024. PMC 3418529. PMID 22943012.
↑ Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016
↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016
↑ Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 02, 2016
↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016
↑ Swerdlow, S.H.; Campo, E.; Harris, N.L.; Jaffe, E.S.; Pileri, S.A.; Stein, H.; Thiele, J.; Vardiman, J.W (2008). "11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma". WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC WHO Classification of Tumours. 2 (4th ed.). IARC. ISBN 9283224310.
↑ [1] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." Am J Surg Pathol. 1999 Oct;23(10):1233-40. PMID: 10524524
↑ Angioimmunoblastic T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52dc/ Accessed on November 27, 2015
↑ Lemonnier F, Couronné L, Parrens M, Jaïs JP, Travert M, Lamant L, Tournillac O, Rousset T, Fabiani B, Cairns RA, Mak T, Bastard C, Bernard OA, de Leval L, Gaulard P (August 2012). "Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters". Blood. 120 (7): 1466–9. doi:10.1182/blood-2012-02-408542. PMID 22760778.
↑ Jha, KunalKishor; Gupta, SureshK; Saluja, Harpreet; Subedi, Nuwadatta (2017). "Peripheral T-cell lymphoma, not otherwise specified". Journal of Family Medicine and Primary Care. 6 (2): 427. doi:10.4103/jfmpc.jfmpc_323_16. ISSN 2249-4863.
↑ ^103.0 ^103.1 ^103.2 Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE; et al. (2009). "Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment". Ann Oncol. 20 (6): 1080–5. doi:10.1093/annonc/mdn751. PMC 4092251. PMID 19237479.
↑ Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y; et al. (2016). "Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases". Am J Surg Pathol. 40 (5): 676–88. doi:10.1097/PAS.0000000000000614. PMID 26872013.
↑ International Non-Hodgkin's Lymphoma Prognostic Factors Project (1993). "A predictive model for aggressive non-Hodgkin's lymphoma". N Engl J Med. 329 (14): 987–94. doi:10.1056/NEJM199309303291402. PMID 8141877.
↑ Saito T, Matsuno Y, Tanosaki R, Watanabe T, Kobayashi Y, Tobinai K (2002). "Gamma delta T-cell neoplasms: a clinicopathological study of 11 cases". Ann Oncol. 13 (11): 1792–8. PMID 12419753.
↑ Boulland ML, Kanavaros P, Wechsler J, Casiraghi O, Gaulard P (1997). "Cytotoxic protein expression in natural killer cell lymphomas and in alpha beta and gamma delta peripheral T-cell lymphomas". J Pathol. 183 (4): 432–9. doi:10.1002/(SICI)1096-9896(199712)183:4<432::AID-PATH942>3.0.CO;2-4. PMID 9496260.
↑ Jonveaux P, Daniel MT, Martel V, Maarek O, Berger R (1996). "Isochromosome 7q and trisomy 8 are consistent primary, non-random chromosomal abnormalities associated with hepatosplenic T gamma/delta lymphoma". Leukemia. 10 (9): 1453–5. PMID 8751461.
↑ Simon A, Peoch M, Casassus P, Deconinck E, Colombat P, Desablens B; et al. (2010). "Upfront VIP-reinforced-ABVD (VIP-rABVD) is not superior to CHOP/21 in newly diagnosed peripheral T cell lymphoma. Results of the randomized phase III trial GOELAMS-LTP95". Br J Haematol. 151 (2): 159–66. doi:10.1111/j.1365-2141.2010.08329.x. PMID 20738307.
↑ Han X, Zhang W, Zhou D, Ruan J, Duan M, Zhu T; et al. (2017). "Autologous stem cell transplantation as frontline strategy for peripheral T-cell lymphoma: A single-centre experience". J Int Med Res. 45 (1): 290–302. doi:10.1177/0300060516676725. PMC 5536587. PMID 28222648.

Template:WikiDoc Sources

[pmid28516671-1] Armitage JO (2017). "The aggressive peripheral T-cell lymphomas: 2017". Am J Hematol. 92 (7): 706–715. doi:10.1002/ajh.24791. PMID 28516671.

[pmid30656034-2] Brandt PH, Rahmat LT, Ali SS (2019). "A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis". Clin Case Rep. 7 (1): 164–169. doi:10.1002/ccr3.1924. PMC 6333078. PMID 30656034.

[pmid29933321-3] Choi Y, Jeon SY, Yoo WH (2018). "Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis". J Clin Rheumatol. doi:10.1097/RHU.0000000000000805. PMID 29933321.

[pmid30596219-4] Gowda L, Foss F (2019). "Hepatosplenic T-Cell Lymphomas". Cancer Treat Res. 176: 185–193. doi:10.1007/978-3-319-99716-2_9. PMID 30596219.

[pmid1698028-5] Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M; et al. (1990). "Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas". Am J Pathol. 137 (3): 617–28. PMC 1877506. PMID 1698028.

[pmid29337025-6] Yabe M, Miranda RN, Medeiros LJ (2018). "Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors". Hum Pathol. 74: 5–16. doi:10.1016/j.humpath.2018.01.005. PMID 29337025.

[pmid14645001-7] Gallamini A, Stelitano C, Calvi R, Bellei M, Mattei D, Vitolo U; et al. (2004). "Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study". Blood. 103 (7): 2474–9. doi:10.1182/blood-2003-09-3080. PMID 14645001.

[pmid8443755-8] Mauch PM, Kalish LA, Kadin M, Coleman CN, Osteen R, Hellman S (March 1993). "Patterns of presentation of Hodgkin disease. Implications for etiology and pathogenesis". Cancer. 71 (6): 2062–71. PMID 8443755.

[pmid4052959-9] Gobbi PG, Cavalli C, Gendarini A, Crema A, Ricevuti G, Federico M, Di Prisco U, Ascari E (December 1985). "Reevaluation of prognostic significance of symptoms in Hodgkin's disease". Cancer. 56 (12): 2874–80. PMID 4052959.

[pmid11238088-10] Buri C, Körner M, Schärli P, Cefai D, Uguccioni M, Mueller C, Laissue JA, Mazzucchelli L (March 2001). "CC chemokines and the receptors CCR3 and CCR5 are differentially expressed in the nonneoplastic leukocytic infiltrates of Hodgkin disease". Blood. 97 (6): 1543–8. PMID 11238088.

[pmid9088951-11] Peh SC, Looi LM, Pallesen G (March 1997). "Epstein-Barr virus (EBV) and Hodgkin's disease in a multi-ethnic population in Malaysia". Histopathology. 30 (3): 227–33. PMID 9088951.

[pmid9110300-12] Andriko JA, Aguilera NS, Nandedkar MA, Abbondanzo SL (April 1997). "Childhood Hodgkin's disease in the United States: an analysis of histologic subtypes and association with Epstein-Barr virus". Mod. Pathol. 10 (4): 366–71. PMID 9110300.

[Colomo2003-13] Colomo, L.; López-Guillermo, A; Perales, M; Rives, S; Martínez, A; Bosch, F; Colomer, D; Falini, B; Montserrat, E; Campo, E (2002). "Clinical impact of the differentiation profile assessed by immunophenotyping in patients with diffuse large B-cell lymphoma". Blood. 101 (1): 78–84. doi:10.1182/blood-2002-04-1286. PMID 12393466.

[Hans2004-14] Hans, C. P.; Weisenburger, D. D.; Greiner, T. C.; Gascoyne, R. D.; Delabie, J; Ott, G; Müller-Hermelink, H. K.; Campo, E; Braziel, R. M.; Jaffe, E. S.; Pan, Z; Farinha, P; Smith, L. M.; Falini, B; Banham, A. H.; Rosenwald, A; Staudt, L. M.; Connors, J. M.; Armitage, J. O.; Chan, W. C. (2004). "Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray". Blood. 103 (1): 275–82. doi:10.1182/blood-2003-05-1545. PMID 14504078.

[Muris2006-15] Muris, JJF; Meijer, Cjlm; Vos, W; Van Krieken, Jhjm; Jiwa, NM; Ossenkoppele, GJ; Oudejans, JJ (2006). "Immunohistochemical profiling based on Bcl-2, CD10 and MUM1 expression improves risk stratification in patients with primary nodal diffuse large B cell lymphoma". The Journal of Pathology. 208 (5): 714–23. doi:10.1002/path.1924. PMID 16400625.

[pmid27271843-16] Korkolopoulou P, Vassilakopoulos T, Milionis V, Ioannou M (2016). "Recent Advances in Aggressive Large B-cell Lymphomas: A Comprehensive Review". Adv Anat Pathol. 23 (4): 202–43. doi:10.1097/PAP.0000000000000117. PMID 27271843.

[17] Swerdlow SH, et al (editors). WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edition. 2008. International Agency for Research on Cancer (IARC), Lyon, Franc

[18] Tilly H, et al. Diffuse large B-cell lymphoma (DLBCL): ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology. 2015; 26: v116-v125

[19] Itziar Salaverria, Cristina Royo, Alejandra Carvajal-Cuenca, Guillem Clot, Alba Navarro, Alejandra Valera, Joo Y. Song, Renata Woroniecka, Grzegorz Rymkiewicz, Wolfram Klapper, Elena M. Hartmann, Pierre Sujobert, Iwona Wlodarska, Judith A. Ferry, Philippe Gaulard, German Ott, Andreas Rosenwald, Armando Lopez-Guillermo, Leticia Quintanilla-Martinez, Nancy L. Harris, Elaine S. Jaffe, Reiner Siebert, Elias Campo & Silvia Bea (2013). "CCND2 rearrangements are the most frequent genetic events in cyclin D1(-) mantle cell lymphoma". Blood. 121 (8): 1394–1402. doi:10.1182/blood-2012-08-452284. PMID 23255553. Unknown parameter |month= ignored (help)

[20] Markus Tiemann, Carsten Schrader, Wolfram Klapper, Martin H. Dreyling, Elias Campo, Andrew Norton, Francoise Berger, Philip Kluin, German Ott, Stephano Pileri, Ennio Pedrinis, Alfred C. Feller, Hartmut Merz, Dirk Janssen, Martin L. Hansmann, Han Krieken, Peter Moller, Harald Stein, Michael Unterhalt, Wolfgang Hiddemann & Reza Parwaresch (2005). "Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network". British journal of haematology. 131 (1): 29–38. doi:10.1111/j.1365-2141.2005.05716.x. PMID 16173960. Unknown parameter |month= ignored (help)

[21] L. H. Argatoff, J. M. Connors, R. J. Klasa, D. E. Horsman & R. D. Gascoyne (1997). "Mantle cell lymphoma: a clinicopathologic study of 80 cases". Blood. 89 (6): 2067–2078. PMID 9058729. Unknown parameter |month= ignored (help)

[22] Markus Tiemann, Carsten Schrader, Wolfram Klapper, Martin H. Dreyling, Elias Campo, Andrew Norton, Francoise Berger, Philip Kluin, German Ott, Stephano Pileri, Ennio Pedrinis, Alfred C. Feller, Hartmut Merz, Dirk Janssen, Martin L. Hansmann, Han Krieken, Peter Moller, Harald Stein, Michael Unterhalt, Wolfgang Hiddemann & Reza Parwaresch (2005). "Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network". British journal of haematology. 131 (1): 29–38. doi:10.1111/j.1365-2141.2005.05716.x. PMID 16173960. Unknown parameter |month= ignored (help)

[23] Julie M. Vose (2017). "Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management". American journal of hematology. 92 (8): 806–813. doi:10.1002/ajh.24797. PMID 28699667. Unknown parameter |month= ignored (help)

[pmid11075849-24] Lin P, Jones D, Dorfman DM, Medeiros LJ (November 2000). "Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement". Am. J. Surg. Pathol. 24 (11): 1480–90. PMID 11075849.

[pmid30410844-25] Javed Z, Hanif F (August 2018). "A Rare Presentation of Precursor B-cell Lymphoblastic Lymphoma in a Child". Cureus. 10 (8): e3238. doi:10.7759/cureus.3238. PMC 6209516. PMID 30410844.

[pmid25548762-26] Kim JY, Om SY, Shin SJ, Kim JE, Yoon DH, Suh C (December 2014). "Case series of precursor B-cell lymphoblastic lymphoma". Blood Res. 49 (4): 270–4. doi:10.5045/br.2014.49.4.270. PMC 4278010. PMID 25548762.

[pmid25176983-27] Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES (2014). "Early lymphoid lesions: conceptual, diagnostic and clinical challenges". Haematologica. 99 (9): 1421–32. doi:10.3324/haematol.2014.107938. PMC 4562530. PMID 25176983.

[pmid11877266-28] Lorsbach RB, Shay-Seymore D, Moore J, Banks PM, Hasserjian RP, Sandlund JT; et al. (2002). "Clinicopathologic analysis of follicular lymphoma occurring in children". Blood. 99 (6): 1959–64. PMID 11877266.

[29] Overview at UMDNJ

[pmid16075463-30] Bosga-Bouwer AG, Haralambieva E, Booman M; et al. (November 2005). "BCL6 alternative translocation breakpoint cluster region associated with follicular lymphoma grade 3B". Genes Chromosomes Cancer. 44 (3): 301–4. doi:10.1002/gcc.20246. PMID 16075463.

[pmid7028244-31] Winberg CD, Nathwani BN, Bearman RM, Rappaport H (1981). "Follicular (nodular) lymphoma during the first two decades of life: a clinicopathologic study of 12 patients". Cancer. 48 (10): 2223–35. PMID 7028244.

[32] Burkitt's Lymphoma. Wikibooks. https://en.wikibooks.org/wiki/Radiation_Oncology/NHL/Burkitt_lymphoma#Pathology Accessed on October,5 2015

[pmid12610094-33] Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L (2003). "Burkitt's lymphoma: new insights into molecular pathogenesis". J. Clin. Pathol. 56 (3): 188–92. PMC 1769902. PMID 12610094. Unknown parameter |month= ignored (help)

[ChuangYe2007-34] Chuang, Shih-Sung; Ye, Hongtao; Du, Ming-Qing; Lu, Chin-Li; Dogan, Ahmet; Hsieh, Pin-Pen; Huang, Wan-Ting; Jung, Yun-Chih (2007). "Histopathology and Immunohistochemistry in Distinguishing Burkitt Lymphoma From Diffuse Large B-Cell Lymphoma With Very High Proliferation Index and With or Without a Starry-Sky Pattern". American Journal of Clinical Pathology. 128 (4): 558–564. doi:10.1309/EQJR3D3V0CCQGP04. ISSN 0002-9173.

[pmid977186-35] Magrath IT, Simon RM (1976). "Immunosuppression in Burkitt's lymphoma. II. Peripheral blood lymphocyte populations related to clinical status". Int J Cancer. 18 (4): 399–408. PMID 977186.

[pmid25856946-36] Basavaraj A, Shinde A, Kulkarni R, Kadam DB, Chugh A (2014). "HIV associated Burkitt's lymphoma". J Assoc Physicians India. 62 (8): 723–7. PMID 25856946.

[RoweFitzsimmons2014-37] Rowe, Martin; Fitzsimmons, Leah; Bell, Andrew I (2014). "Epstein-Barr virus and Burkitt lymphoma". Chinese Journal of Cancer. doi:10.5732/cjc.014.10190. ISSN 1000-467X.

[pmid30290813-38] Ekemen S, Uzay A, Bassullu N, Dikicioglu-Cetin E, Matsuda K, Ince U; et al. (2018). "Does it take three to tango? An unsuspected multimorbidity of CD8+ T cell lymphoproliferative disorder, malaria, and EBV infection". Malar J. 17 (1): 349. doi:10.1186/s12936-018-2497-9. PMC 6173833. PMID 30290813.

[pmid26437030-39] Kretzmer H, Bernhart SH, Wang W, Haake A, Weniger MA, Bergmann AK; et al. (2015). "DNA methylome analysis in Burkitt and follicular lymphomas identifies differentially methylated regions linked to somatic mutation and transcriptional control". Nat Genet. 47 (11): 1316–1325. doi:10.1038/ng.3413. PMC 5444523. PMID 26437030.

[KleinTu2001-40] Klein, Ulf; Tu, Yuhai; Stolovitzky, Gustavo A.; Mattioli, Michela; Cattoretti, Giorgio; Husson, Hervé; Freedman, Arnold; Inghirami, Giorgio; Cro, Lilla; Baldini, Luca; Neri, Antonino; Califano, Andrea; Dalla-Favera, Riccardo (2001). "Gene Expression Profiling of B Cell Chronic Lymphocytic Leukemia Reveals a Homogeneous Phenotype Related to Memory B Cells". The Journal of Experimental Medicine. 194 (11): 1625–1638. doi:10.1084/jem.194.11.1625. ISSN 0022-1007.

[sympmalylymmnoingastry-41] Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016

[riskfactorsmaltlymphoma1-42] Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016

[KinkadeEsan2015-43] Kinkade, Zoe; Esan, Olukemi A.; Rosado, Flavia G.; Craig, Michael; Vos, Jeffrey A. (2015). "Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report". Diagnostic Pathology. 10 (1). doi:10.1186/s13000-015-0353-6. ISSN 1746-1596.

[sympromaltlymphoma2-44] Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016

[sympromaltlymphoma22-45] Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016

[sympromaltlymphoma1-46] Signs and symptoms of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 28, 2016

[nongastricorbitlymphomasym1-47] Clinical presentation of orbital lymphoma. Dr Craig Hacking and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/orbital-lymphoma. Accessed on January 28, 2016

[sympmalylymmnoingastry2-48] Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016

[49] Taal, B G; Boot, H; van Heerde, P; de Jong, D; Hart, A A; Burgers, J M (1 October 1996). "Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept". Gut. 39 (4): 556–561. doi:10.1136/gut.39.4.556.

[pmid16950858-50] Bacon CM, Du MQ, Dogan A (2007). "Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists". J Clin Pathol. 60 (4): 361–72. doi:10.1136/jcp.2005.031146. PMC 2001121. PMID 16950858.

[pmid11337382-51] Hernández JM, García JL, Gutiérrez NC, Mollejo M, Martínez-Climent JA, Flores T, González MB, Piris MA, San Miguel JF (May 2001). "Novel genomic imbalances in B-cell splenic marginal zone lymphomas revealed by comparative genomic hybridization and cytogenetics". Am. J. Pathol. 158 (5): 1843–50. doi:10.1016/S0002-9440(10)64140-5. PMC 1891967. PMID 11337382.

[pmid15642391-52] Andersen CL, Gruszka-Westwood A, Atkinson S, Matutes E, Catovsky D, Pedersen RK, Pedersen BB, Pulczynski S, Hokland P, Jacobsen E, Koch J (January 2005). "Recurrent genomic imbalances in B-cell splenic marginal-zone lymphoma revealed by comparative genomic hybridization". Cancer Genet. Cytogenet. 156 (2): 122–8. doi:10.1016/j.cancergencyto.2004.04.026. PMID 15642391.

[pmid20479288-53] Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F (September 2010). "Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group". Blood. 116 (9): 1479–88. doi:10.1182/blood-2010-02-267476. PMID 20479288.

[54] Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on December 22, 2015

[pmid12916862-55] Weng WK, Levy S (July 2003). "Hepatitis C virus (HCV) and lymphomagenesis". Leuk. Lymphoma. 44 (7): 1113–20. doi:10.1080/1042819031000076972. PMID 12916862.

[pmid11739181-56] Quinn ER, Chan CH, Hadlock KG, Foung SK, Flint M, Levy S (December 2001). "The B-cell receptor of a hepatitis C virus (HCV)-associated non-Hodgkin lymphoma binds the viral E2 envelope protein, implicating HCV in lymphomagenesis". Blood. 98 (13): 3745–9. PMID 11739181.

[pmid20530156-57] Chuang SS, Liao YL, Chang ST, Hsieh YC, Kuo SY, Lu CL, Hwang WS, Lin IH, Tsao CJ, Huang WT (July 2010). "Hepatitis C virus infection is significantly associated with malignant lymphoma in Taiwan, particularly with nodal and splenic marginal zone lymphomas". J. Clin. Pathol. 63 (7): 595–8. doi:10.1136/jcp.2010.076810. PMID 20530156.

[SpinaKhiabanian2016-58] Spina, V.; Khiabanian, H.; Messina, M.; Monti, S.; Cascione, L.; Bruscaggin, A.; Spaccarotella, E.; Holmes, A. B.; Arcaini, L.; Lucioni, M.; Tabbo, F.; Zairis, S.; Diop, F.; Cerri, M.; Chiaretti, S.; Marasca, R.; Ponzoni, M.; Deaglio, S.; Ramponi, A.; Tiacci, E.; Pasqualucci, L.; Paulli, M.; Falini, B.; Inghirami, G.; Bertoni, F.; Foa, R.; Rabadan, R.; Gaidano, G.; Rossi, D. (2016). "The genetics of nodal marginal zone lymphoma". Blood. 128 (10): 1362–1373. doi:10.1182/blood-2016-02-696757. ISSN 0006-4971.

[canadiancancer-59] Nodal marginal zone lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/nodal-marginal-zone-lymphoma/?region=nb Accessed on March 4, 2016

[60] Steven P. Treon, Lian Xu, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Patricia Sheehy, Robert J. Manning, Christopher J. Patterson, Christina Tripsas, Luca Arcaini, Geraldine S. Pinkus, Scott J. Rodig, Aliyah R. Sohani, Nancy Lee Harris, Jason M. Laramie, Donald A. Skifter, Stephen E. Lincoln & Zachary R. Hunter (2012). "MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia". The New England journal of medicine. 367 (9): 826–833. doi:10.1056/NEJMoa1200710. PMID 22931316. Unknown parameter |month= ignored (help)

[AC-61] Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT (2014). "Renal MALT lymphoma associated with Waldenström macroglobulinemia". J. Formos. Med. Assoc. 113 (4): 255–7. doi:10.1016/j.jfma.2011.02.007. PMID 24685302.

[AC2-62] Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT (2014). "Renal MALT lymphoma associated with Waldenström macroglobulinemia". J. Formos. Med. Assoc. 113 (4): 255–7. doi:10.1016/j.jfma.2011.02.007. PMID 24685302.

[pmid11736938-63] García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A; et al. (2001). "Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases". Br J Haematol. 115 (3): 575–82. PMID 11736938.

[pmid12720138-64] Merlini G, Baldini L, Broglia C, Comelli M, Goldaniga M, Palladini G; et al. (2003). "Prognostic factors in symptomatic Waldenstrom's macroglobulinemia". Semin Oncol. 30 (2): 211–5. doi:10.1053/sonc.2003.50064. PMID 12720138.

[pmid16990106-65] Matutes E (October 2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol. Oncol. Clin. North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.

[pmid169901062-66] Matutes E (October 2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol. Oncol. Clin. North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.

[pmid22210875-67] Xi L, Arons E, Navarro W, Calvo KR, Stetler-Stevenson M, Raffeld M, Kreitman RJ (April 2012). "Both variant and IGHV4-34-expressing hairy cell leukemia lack the BRAF V600E mutation". Blood. 119 (14): 3330–2. doi:10.1182/blood-2011-09-379339. PMC 3321859. PMID 22210875.

[pmid21910720-68] Boyd EM, Bench AJ, van 't Veer MB, Wright P, Bloxham DM, Follows GA, Scott MA (December 2011). "High resolution melting analysis for detection of BRAF exon 15 mutations in hairy cell leukaemia and other lymphoid malignancies". Br. J. Haematol. 155 (5): 609–12. doi:10.1111/j.1365-2141.2011.08868.x. PMID 21910720.

[pmid15710587-69] Forconi F, Raspadori D, Lenoci M, Lauria F (February 2005). "Absence of surface CD27 distinguishes hairy cell leukemia from other leukemic B-cell malignancies". Haematologica. 90 (2): 266–8. PMID 15710587.

[pmid28892922-70] Shelly D, Gujral S (2017). "Early T-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma: Immunohistochemical Evaluation of Four Lymph Node Biopsies". J Clin Diagn Res. 11 (7): EL01–EL02. doi:10.7860/JCDR/2017/29352.10164. PMC 5583929. PMID 28892922.

[pmid26276771-71] You MJ, Medeiros LJ, Hsi ED (2015). "T-lymphoblastic leukemia/lymphoma". Am J Clin Pathol. 144 (3): 411–22. doi:10.1309/AJCPMF03LVSBLHPJ. PMID 26276771.

[Rose2004-72] Rose, M. G. (2004). "T-Cell Large Granular Lymphocyte Leukemia and Related Disorders". The Oncologist. 9 (3): 247–258. doi:10.1634/theoncologist.9-3-247. ISSN 1083-7159.

[pmid22800517-73] Liu EB, Chen HS, Zhang PH, Li ZQ, Sun Q, Yang QY; et al. (2012). "[Hematopathologic features of T-cell large granular lymphocytic leukemia]". Zhonghua Bing Li Xue Za Zhi. 41 (4): 229–33. doi:10.3760/cma.j.issn.0529-5807.2012.04.004. PMID 22800517.

[pmid15958859-74] Osuji N, Matutes E, Catovsky D, Lampert I, Wotherspoon A (2005). "Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review". Am J Surg Pathol. 29 (7): 935–41. PMID 15958859.

[pmid23382603-75] Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.

[pmid18042693-76] Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.

[pmid180426932-77] Matutes E (2007) Adult T-cell leukaemia/lymphoma. J Clin Pathol 60 (12):1373-7. DOI:10.1136/jcp.2007.052456 PMID: 18042693

[pmid1751370-78] Shimoyama M (1991). "Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87)". Br J Haematol. 79 (3): 428–37. PMID 1751370.

[Al-AhmadMaertens2017-79] Al-Ahmad, Selma; Maertens, Vincent; Libeer, Christophe; Schelfhout, Vera; Vanhoenacker, Filip; Boeckx, Nancy; Vandevenne, Marleen (2017). "The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature". Acta Clinica Belgica. 72 (6): 454–460. doi:10.1080/17843286.2017.1312057. ISSN 1784-3286.

[pmid25197351-80] Yu L, Yan LL, Yang SJ (2014). "Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature". Int J Clin Exp Pathol. 7 (8): 4806–16. PMC 4152041. PMID 25197351.

[pmid28652989-81] Campos FPF, Zerbini MCN, Felipe-Silva A, Simões AB, Lovisolo SM, da Fonseca LG; et al. (2014). "Unusual clinical presentation of anaplastic large cell lymphoma". Autops Case Rep. 4 (1): 21–27. doi:10.4322/acr.2014.004. PMC 5470561. PMID 28652989.

[pmid17965727-82] Watanabe M, Ogawa Y, Itoh K; et al. (January 2008). "Hypomethylation of CD30 CpG islands with aberrant JunB expression drives CD30 induction in Hodgkin lymphoma and anaplastic large cell lymphoma". Lab. Invest. 88 (1): 48–57. doi:10.1038/labinvest.3700696. PMID 17965727.

[FossGirardi2017-83] Foss, Francine M.; Girardi, Michael (2017). "Mycosis Fungoides and Sezary Syndrome". Hematology/Oncology Clinics of North America. 31 (2): 297–315. doi:10.1016/j.hoc.2016.11.008. ISSN 0889-8588.

[CampbellClark2010-84] Campbell, J. J.; Clark, R. A.; Watanabe, R.; Kupper, T. S. (2010). "Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors". Blood. 116 (5): 767–771. doi:10.1182/blood-2009-11-251926. ISSN 0006-4971.

[VonderheidBernengo2002-85] Vonderheid, Eric C.; Bernengo, Maria Grazia; Burg, Günter; Duvic, Madeleine; Heald, Peter; Laroche, Liliane; Olsen, Elise; Pittelkow, Mark; Russell-Jones, Robin; Takigawa, Masahiro; Willemze, Rein (2002). "Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas". Journal of the American Academy of Dermatology. 46 (1): 95–106. doi:10.1067/mjd.2002.118538. ISSN 0190-9622.

[seer.cancer.gov-86] Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016

[seer.cancer.gov2-87] Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016

[pmid191959752-88] Parveen Z, Thompson K (2009). "Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas". Arch Pathol Lab Med. 133 (2): 303–8. doi:10.1043/1543-2165-133.2.303. PMID 19195975.

[pmid28058028-89] van de Meeberg MM, Derikx LA, Sinnige HA, Nooijen P, Schipper DL, Nissen LH (2016). "Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy". World J Gastroenterol. 22 (47): 10465–10470. doi:10.3748/wjg.v22.i47.10465. PMC 5175260. PMID 28058028.

[ShiWang2015-90] Shi, Yang; Wang, Endi (2015). "Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer–Cell Neoplasms". Archives of Pathology & Laboratory Medicine. 139 (9): 1173–1180. doi:10.5858/arpa.2014-0079-RS. ISSN 0003-9985.

[pmid22379294-91] Alsohaibani FI, Abdulla MA, Fagih MM (2011). "Hepatosplenic T-cell lymphoma". Indian J Hematol Blood Transfus. 27 (1): 39–42. doi:10.1007/s12288-010-0051-1. PMC 3102508. PMID 22379294.

[DelabieHolte2011-92] Delabie, J.; Holte, H.; Vose, J. M.; Ullrich, F.; Jaffe, E. S.; Savage, K. J.; Connors, J. M.; Rimsza, L.; Harris, N. L.; Muller-Hermelink, K.; Rudiger, T.; Coiffier, B.; Gascoyne, R. D.; Berger, F.; Tobinai, K.; Au, W. Y.; Liang, R.; Montserrat, E.; Hochberg, E. P.; Pileri, S.; Federico, M.; Nathwani, B.; Armitage, J. O.; Weisenburger, D. D. (2011). "Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project". Blood. 118 (1): 148–155. doi:10.1182/blood-2011-02-335216. ISSN 0006-4971.

[pmid22943012-93] Bautista-Quach MA, Ake CD, Chen M, Wang J (September 2012). "Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features". J Gastrointest Oncol. 3 (3): 209–25. doi:10.3978/j.issn.2078-6891.2012.024. PMC 3418529. PMID 22943012.

[cancer.gov-94] Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016

[Hindawi-95] Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016

[cancer.gov2-96] Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 02, 2016

[Hindawi2-97] Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016

[who1-98] Swerdlow, S.H.; Campo, E.; Harris, N.L.; Jaffe, E.S.; Pileri, S.A.; Stein, H.; Thiele, J.; Vardiman, J.W (2008). "11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma". WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC WHO Classification of Tumours. 2 (4th ed.). IARC. ISBN 9283224310.

[quin1-99] [1] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." Am J Surg Pathol. 1999 Oct;23(10):1233-40. PMID: 10524524

[seer-100] Angioimmunoblastic T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52dc/ Accessed on November 27, 2015

[pmid22760778-101] Lemonnier F, Couronné L, Parrens M, Jaïs JP, Travert M, Lamant L, Tournillac O, Rousset T, Fabiani B, Cairns RA, Mak T, Bastard C, Bernard OA, de Leval L, Gaulard P (August 2012). "Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters". Blood. 120 (7): 1466–9. doi:10.1182/blood-2012-02-408542. PMID 22760778.

[JhaGupta2017-102] Jha, KunalKishor; Gupta, SureshK; Saluja, Harpreet; Subedi, Nuwadatta (2017). "Peripheral T-cell lymphoma, not otherwise specified". Journal of Family Medicine and Primary Care. 6 (2): 427. doi:10.4103/jfmpc.jfmpc_323_16. ISSN 2249-4863.

[pmid19237479-103] 103.0 ^103.1 ^103.2 Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE; et al. (2009). "Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment". Ann Oncol. 20 (6): 1080–5. doi:10.1093/annonc/mdn751. PMC 4092251. PMID 19237479.

[pmid26872013-104] Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y; et al. (2016). "Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases". Am J Surg Pathol. 40 (5): 676–88. doi:10.1097/PAS.0000000000000614. PMID 26872013.

[pmid8141877-105] International Non-Hodgkin's Lymphoma Prognostic Factors Project (1993). "A predictive model for aggressive non-Hodgkin's lymphoma". N Engl J Med. 329 (14): 987–94. doi:10.1056/NEJM199309303291402. PMID 8141877.

[pmid12419753-106] Saito T, Matsuno Y, Tanosaki R, Watanabe T, Kobayashi Y, Tobinai K (2002). "Gamma delta T-cell neoplasms: a clinicopathological study of 11 cases". Ann Oncol. 13 (11): 1792–8. PMID 12419753.

[pmid9496260-107] Boulland ML, Kanavaros P, Wechsler J, Casiraghi O, Gaulard P (1997). "Cytotoxic protein expression in natural killer cell lymphomas and in alpha beta and gamma delta peripheral T-cell lymphomas". J Pathol. 183 (4): 432–9. doi:10.1002/(SICI)1096-9896(199712)183:4<432::AID-PATH942>3.0.CO;2-4. PMID 9496260.

[pmid8751461-108] Jonveaux P, Daniel MT, Martel V, Maarek O, Berger R (1996). "Isochromosome 7q and trisomy 8 are consistent primary, non-random chromosomal abnormalities associated with hepatosplenic T gamma/delta lymphoma". Leukemia. 10 (9): 1453–5. PMID 8751461.

[pmid20738307-109] Simon A, Peoch M, Casassus P, Deconinck E, Colombat P, Desablens B; et al. (2010). "Upfront VIP-reinforced-ABVD (VIP-rABVD) is not superior to CHOP/21 in newly diagnosed peripheral T cell lymphoma. Results of the randomized phase III trial GOELAMS-LTP95". Br J Haematol. 151 (2): 159–66. doi:10.1111/j.1365-2141.2010.08329.x. PMID 20738307.

[pmid28222648-110] Han X, Zhang W, Zhou D, Ruan J, Duan M, Zhu T; et al. (2017). "Autologous stem cell transplantation as frontline strategy for peripheral T-cell lymphoma: A single-centre experience". J Int Med Res. 45 (1): 290–302. doi:10.1177/0300060516676725. PMC 5536587. PMID 28222648.

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@@ Line 1: / Line 1: @@
 __NOTOC__
 '''For patient information, click [[Hepatosplenic T cell lymphoma (patient information)|here]]'''
 {{Hepatosplenic T cell lymphoma}}
-{{CMG}}; {{AE}} {{AS}}
-'''''Synonyms and Keywords:''''' Hepatosplenic γδ T-cell lymphoma, Hepatosplenic gamma delta T-cell lymphoma
+{{CMG}}; {{AE}}{{JSS}}
+'''''Synonyms and Keywords:''''' Hepatosplenic γδ T-cell lymphoma, Hepatosplenic gamma delta T-cell lymphoma, HSTCL
+==Overview==
+Hepatosplenic T cell lymphoma is a rare type of non Hodgkins lymphoma that occurs in states of immunosupression such as post organ transplant and treatment of inflammatory bowel disease. Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990''.'' It is also known as "gamma-delta" hepatosplenic T-cell lymphoma. The [[lymphoma]] is characterized by malignant [[T-cell proliferation]] in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow. The incidence of hepatosplenic T cell lymphoma is 0.3 per 100000 individuals per year. If left untreated, patients can develop liver failure, pancytopenia or spleen rupture. Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic T-cell lymphoma. Chemotherapy including CHOP is the mainstay of the treatment along with [[Bone marrow transplantation|bone marrow transplant]] and radiation therapy.
+==Historical Perspective==
+Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990''.''
+==Classification==
+There is no established system for the classification of hepatosplenic T cell lymphoma.
+==Pathophysiology==
+* Hepatosplenic T-cell lymphoma is a [[T-cell lymphoma classification|peripheral T-cell lymphoma]], a type of [[Non-Hodgkin lymphoma|non Hodgkin's lymphoma]].<ref name="pmid28516671">{{cite journal| author=Armitage JO| title=The aggressive peripheral T-cell lymphomas: 2017. | journal=Am J Hematol | year= 2017 | volume= 92 | issue= 7 | pages= 706-715 | pmid=28516671 | doi=10.1002/ajh.24791 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28516671  }} </ref>
+* It is also known as "gamma-delta hepatosplenic T-cell lymphoma".<ref name="pmid30656034">{{cite journal| author=Brandt PH, Rahmat LT, Ali SS| title=A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis. | journal=Clin Case Rep | year= 2019 | volume= 7 | issue= 1 | pages= 164-169 | pmid=30656034 | doi=10.1002/ccr3.1924 | pmc=6333078 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30656034  }} </ref>
+* It usually occurs in young men with history of [[immunosuppression]] including solid [[Organ transplant|organ transplantation]].<ref name="pmid29933321">{{cite journal| author=Choi Y, Jeon SY, Yoo WH| title=Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis. | journal=J Clin Rheumatol | year= 2018 | volume=  | issue=  | pages=  | pmid=29933321 | doi=10.1097/RHU.0000000000000805 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29933321  }} </ref>
+* Patients with [[inflammatory bowel disease]] receiving [[immunosuppressants]] and [[Tumor necrosis factor-alpha|anti-tumor necrosis factor-α]] agent are also at risk for developing hepatosplenic T-cell lymphoma.
+* The T-cell  receptor consists of either a gamma delta or alpha-beta entity on their cell surface which are a part of the [[Innate intelligence|innate]] immune system.<ref name="pmid30596219">{{cite journal| author=Gowda L, Foss F| title=Hepatosplenic T-Cell Lymphomas. | journal=Cancer Treat Res | year= 2019 | volume= 176 | issue=  | pages= 185-193 | pmid=30596219 | doi=10.1007/978-3-319-99716-2_9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30596219  }} </ref><ref name="pmid1698028">{{cite journal| author=Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M et al.| title=Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas. | journal=Am J Pathol | year= 1990 | volume= 137 | issue= 3 | pages= 617-28 | pmid=1698028 | doi= | pmc=1877506 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1698028  }} </ref>
+* Gamma delta T cells represent the first line of defense against [[Bacteria|bacterial]] [[Peptide|peptides]], such as [[Heat shock protein|heat-shock proteins]].
+* Gamma delta T cells are [[CD4]] and [[CD8]] negative, but [[CD56]] positive which is [[Natural Killer T cell|NK cell marker]].
+* Gamma delta cells respond to a stimulus and are responsible for [[lymphokine]] production and proliferation.
+* Gamma delta cells are predominantly located in the [[spleen]], [[liver]] sinusoids and [[Intestine|intestinal]] epithelium.
+* 75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
+* Chronic antigen stimulation in states of immunosuppression is responsible for the development of the [[lymphoma]].
+* [[Isochromosome]] of the long arm of [[chromosome 7]] is a genetic abnormality described in hepatosplenic T cell lymphoma. It could also be in association with [[trisomy]] 8 and a loss of a [[sex chromosome]].
+* Mutations in ''[[SETD2]], [[INO80 complex subunit E|INO80]], TET3'' and ''[[STAT5B]]'' occur exclusively in hepatosplenic T cell lymphoma as compared to other T and B cell lymphoma types.
+* The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
+* Splenic involvement is characterized by diffuse involvement of the [[red pulp]] with small-to-medium-sized [[atypical lymphocytes]].
+* The atypical lymphocytes are present within the cords and sinuses of the red pulp.
+* There occurs a complete loss of the [[white pulp]].
+* The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.
+* The bone marrow is characterized by neoplastic cells in the sinusoids.
+* Bone marrow infiltration results in [[pancytopenia]].
+* The lymphoma cells are typically [[CD2|CD2+]], [[CD3 (immunology)|CD3+]], CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
+* It manifests as [[hepatosplenomegaly]] without peripheral [[lymphadenopathy]].
+* [[Pancytopenia]] and abnormal liver functions are the laboratory findings.
+* Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.
+==Causes==
+Common causes of hepatosplenic  T cell lymphoma are: <ref name="pmid29337025">{{cite journal| author=Yabe M, Miranda RN, Medeiros LJ| title=Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors. | journal=Hum Pathol | year= 2018 | volume= 74 | issue=  | pages= 5-16 | pmid=29337025 | doi=10.1016/j.humpath.2018.01.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29337025  }} </ref><ref name="pmid14645001">{{cite journal| author=Gallamini A, Stelitano C, Calvi R, Bellei M, Mattei D, Vitolo U et al.| title=Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study. | journal=Blood | year= 2004 | volume= 103 | issue= 7 | pages= 2474-9 | pmid=14645001 | doi=10.1182/blood-2003-09-3080 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14645001  }} </ref>
+* [[Inflammatory bowel disease]]
+* [[Organ transplant]] patients (receiver)
+* [[Immunosuppresive drug|Immunosuppressive]] medications
+* [[Thiopurine|Thiopurines]]
+* [[Infliximab]]
+* [[Cyclophosphamide]]
+* [[Vincristine sulfate|Vincristine]]
+* [[Doxorubicin hydrochloride|Doxorubicin]]
+==Differentiating hepatosplenic T cell lymphoma from Other Diseases==
+Differential diagnosis for the lymphoma is based on the below table:
+{| class="wikitable"
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating diagnosis of Lymphoma
+! colspan="5" style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptoms
+! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Signs
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Additional Findings
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Fever
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Rash
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diarrhea
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Abdominal pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Weight loss
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Painful lymphadenopathy
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Hepatosplenomegaly
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Arthritis
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |Lab Findings
+|-
+| style="background:#DCDCDC;" align="center" + |[[Lymphoma]]
+| +
+|–
+|–
+| +
+| +
+|–
+| +
+|–
+|Increase [[ESR]], increased [[LDH]]
+|[[Night sweats]], constant fatigue
+|-
+| style="background:#DCDCDC;" align="center" + |[[Brucellosis]]
+| +
+| +
+|–
+| +
+| +
+| +
+| +
+| +
+|[[Lymphocytosis|Relative lymphocytosis]]
+|[[Night sweats]], often with characteristic smell, likened to wet hay
+|-
+| style="background:#DCDCDC;" align="center" + |[[Typhoid fever]]
+| +
+| +
+|–
+| +
+|–
+|–
+| +
+| +
+|Decreased [[hemoglobin]]
+|Incremental increase in temperature initially and than sustained [[fever]] as high as 40°C (104°F)
+|-
+| style="background:#DCDCDC;" align="center" + |[[Malaria]]
+| +
+|–
+| +
+| +
+|–
+|–
+| +
+| +
+|Microcytosis,
+elevated [[LDH]]
+|"Tertian" fever: paroxysms occur every second day
+|-
+| style="background:#DCDCDC;" align="center" + |[[Tuberculosis]]
+| +
+| +
+|–
+| +
+| +
+| +
+|–
+| +
+|Mild normocytic [[anemia]], [[hyponatremia]], and
+[[hypercalcemia]]
+|[[Night sweats]], constant fatigue
+|-
+| style="background:#DCDCDC;" align="center" + |[[Mumps]]
+| +
+|–
+|–
+|–
+|–
+| +
+|–
+|–
+|[[Lymphocytosis|Relative lymphocytosis]], serum [[amylase]]<nowiki/>elevated
+|[[Parotid gland|Parotid]]<nowiki/>swelling/tenderness
+|-
+| style="background:#DCDCDC;" align="center" + |[[Rheumatoid arthritis]]
+|–
+| +
+|–
+|–
+|–
+|–
+|–
+| +
+|[[ESR]] and [[CRP]] elevated, positive [[rheumatoid factor]]
+|Morning stiffness
+|-
+| style="background:#DCDCDC;" align="center" + |[[SLE]]
+|–
+| +
+|–
+| +
+| +
+|–
+|–
+| +
+|[[ESR]] and [[CRP]] elevated, positive [[ANA]]
+|[[Fatigue]]
+|-
+| style="background:#DCDCDC;" align="center" + |[[Human Immunodeficiency Virus|HIV]]
+|–
+|–
+|–
+| +
+| +
+| +
+|–
+| +
+|Leukopenia
+|Constant fatigue
+|}
+Differentiating different types of T-cell Non-Hodgkin lymphoma. The gold standard for differentiation different types of Non-Hodgkin lymphoma is biopsy.
+{|
+! colspan="2" rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
+! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
+! colspan="6" rowspan="2" style="background:#4479BA; color: #FFFFFF;" + |Clinical manifestations
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" |Paraclinical findings
+! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
+|-
+! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab findings
+|-
+! colspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Symptoms
+! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Signs
+! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunochemistry
+! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
+|-
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Constitutional symptoms
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Rash
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Abdominal
+pain
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Diarrhea
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mass
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Other
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" color: #FFFFFF;" + |[[Hodgkin's lymphoma|Hodgkin's Lymphoma]]<br><ref name="pmid8443755">{{cite journal |vauthors=Mauch PM, Kalish LA, Kadin M, Coleman CN, Osteen R, Hellman S |title=Patterns of presentation of Hodgkin disease. Implications for etiology and pathogenesis |journal=Cancer |volume=71 |issue=6 |pages=2062–71 |date=March 1993 |pmid=8443755 |doi= |url=}}</ref><ref name="pmid4052959">{{cite journal |vauthors=Gobbi PG, Cavalli C, Gendarini A, Crema A, Ricevuti G, Federico M, Di Prisco U, Ascari E |title=Reevaluation of prognostic significance of symptoms in Hodgkin's disease |journal=Cancer |volume=56 |issue=12 |pages=2874–80 |date=December 1985 |pmid=4052959 |doi= |url=}}</ref><ref name="pmid11238088">{{cite journal |vauthors=Buri C, Körner M, Schärli P, Cefai D, Uguccioni M, Mueller C, Laissue JA, Mazzucchelli L |title=CC chemokines and the receptors CCR3 and CCR5 are differentially expressed in the nonneoplastic leukocytic infiltrates of Hodgkin disease |journal=Blood |volume=97 |issue=6 |pages=1543–8 |date=March 2001 |pmid=11238088 |doi= |url=}}</ref><ref name="pmid9088951">{{cite journal |vauthors=Peh SC, Looi LM, Pallesen G |title=Epstein-Barr virus (EBV) and Hodgkin's disease in a multi-ethnic population in Malaysia |journal=Histopathology |volume=30 |issue=3 |pages=227–33 |date=March 1997 |pmid=9088951 |doi= |url=}}</ref><ref name="pmid9110300">{{cite journal |vauthors=Andriko JA, Aguilera NS, Nandedkar MA, Abbondanzo SL |title=Childhood Hodgkin's disease in the United States: an analysis of histologic subtypes and association with Epstein-Barr virus |journal=Mod. Pathol. |volume=10 |issue=4 |pages=366–71 |date=April 1997 |pmid=9110300 |doi= |url=}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Activation of (NF-kB)
+* Mutations in JAK-STAT pathway
+* NF-kB leads to activation of many genes like:
+**[[ICAM-1|(ICAM-1) gene]]
+**[[Granulocyte macrophage colony stimulating factor|GM-CSF gene]]
+**[[Interleukin 6|IL-6 gene]]
+**IKBA gene
+**Gain of function mutation of the MAP3K14 gene
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]] ([[Pel-Ebstein fever]]): persistent temperature >38°C (>100.4°F)
+*Drenching [[night sweats]]
+*Unexplained weight loss >10% of total body weight within the past 6 months
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + |±
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* Painless  [[lymphadenopathy]] with a rubbery consistency.
+* Mostly above the diaphram
+| align="left" style="background:#F5F5F5;" + |
+* [[Itch|Pruritus]]
+*Pain in lymph nodes after drinking alcohol
+*Skin blushing or [[flushing]]
+*[[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |
+:Nodular lymphocyte predominant
+:* [[CD19]]+
+:* [[CD20]]+
+:* [[CD22]]+
+:* [[CD15]]-
+:* [[CD30]] Classical Hodgkin's lymphoma
+:* [[CD15]]+
+:* [[CD30]]+
+:* [[CD20]]-
+:* [[CD45]]-
+*
+| align="left" style="background:#F5F5F5;" + |
+* Effacement of the lymph node architecture by [[Reed-Sternberg cells]] .
+* [[Reed-Sternberg cell|Reed-Sternberg]] cell is a large often bi-nucleated cells with prominent nucleoli and an unusual  immuno phenotype.
+| align="left" style="background:#F5F5F5;" + |
+* [[EBV infection]]
+|-
+! colspan="12" align="center" style="background:#7d7d7d; color: #FFFFFF;" + |Non-Hodgkin's Lymphoma (B Cell Lymphoma)
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Diffuse large B cell lymphoma]]<br><ref name="Colomo2003">{{cite journal |doi=10.1182/blood-2002-04-1286 |pmid=12393466 |title=Clinical impact of the differentiation profile assessed by immunophenotyping in patients with diffuse large B-cell lymphoma |journal=Blood |volume=101 |issue=1 |pages=78–84 |year=2002 |last1=Colomo |first1=L. |last2=López-Guillermo |first2=A |last3=Perales |first3=M |last4=Rives |first4=S |last5=Martínez |first5=A |last6=Bosch |first6=F |last7=Colomer |first7=D |last8=Falini |first8=B |last9=Montserrat |first9=E |last10=Campo |first10=E }}</ref><ref name="Hans2004">{{cite journal |doi=10.1182/blood-2003-05-1545 |pmid=14504078 |title=Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray |journal=Blood |volume=103 |issue=1 |pages=275–82 |year=2004 |last1=Hans |first1=C. P. |last2=Weisenburger |first2=D. D. |last3=Greiner |first3=T. C. |last4=Gascoyne |first4=R. D. |last5=Delabie |first5=J |last6=Ott |first6=G |last7=Müller-Hermelink |first7=H. K. |last8=Campo |first8=E |last9=Braziel |first9=R. M. |last10=Jaffe |first10=E. S. |last11=Pan |first11=Z |last12=Farinha |first12=P |last13=Smith |first13=L. M. |last14=Falini |first14=B |last15=Banham |first15=A. H. |last16=Rosenwald |first16=A |last17=Staudt |first17=L. M. |last18=Connors |first18=J. M. |last19=Armitage |first19=J. O. |last20=Chan |first20=W. C. }}</ref><ref name="Muris2006">{{cite journal |doi=10.1002/path.1924 |pmid=16400625 |title=Immunohistochemical profiling based on Bcl-2, CD10 and MUM1 expression improves risk stratification in patients with primary nodal diffuse large B cell lymphoma |journal=The Journal of Pathology |volume=208 |issue=5 |pages=714–23 |year=2006 |last1=Muris |first1=JJF |last2=Meijer |first2=Cjlm |last3=Vos |first3=W |last4=Van Krieken |first4=Jhjm |last5=Jiwa |first5=NM |last6=Ossenkoppele |first6=GJ |last7=Oudejans |first7=JJ }}</ref><ref name="pmid27271843">{{cite journal| author=Korkolopoulou P, Vassilakopoulos T, Milionis V, Ioannou M| title=Recent Advances in Aggressive Large B-cell Lymphomas: A Comprehensive Review. | journal=Adv Anat Pathol | year= 2016 | volume= 23 | issue= 4 | pages= 202-43 | pmid=27271843 | doi=10.1097/PAP.0000000000000117 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27271843  }} </ref><ref>Swerdlow SH, et al (editors). WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edition. 2008. International Agency for Research on Cancer (IARC), Lyon, Franc</ref><ref>Tilly H, et al. Diffuse large B-cell lymphoma (DLBCL): ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology. 2015; 26: v116-v125</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell Lymphoma
+* 8q24/''MYC'' [[translocations]]
+* Hypermutated immunoglobulin genes
+* Mutation of ''BCL6'', ''MYC'', ''[[PAX5]]'', ''[[PIM1]]'', ''[[RhoH]]'' /''TTFn'', ''[[TP53]]'' genes
+* Translocations involving ''c-MYC'', ''BCL6'', and ''IgH'' gene.
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Night sweats]]
+* [[Weight loss]]
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="left" style="background:#F5F5F5;" + |
+* Painless rapidly enlarging [[lymph nodes]]
+| align="left" style="background:#F5F5F5;" + |
+* Extranodal involvement of [[GI tract]], [[lungs]], and [[CNS]]
+| align="left" style="background:#F5F5F5;" + |
+* CD20
+* CD3
+* CD5
+* CD45
+* CD10
+* BCL-2
+* BCl-6
+* MYC
+* IRF-4/ MUM-1
+* Ki-67
+| align="left" style="background:#F5F5F5;" + |
+'''Centroblastic'''
+*Medium-to-large-sized [[Lymphocyte|lymphocytes]]
+*Monomorphic
+'''Immunoblastic'''
+*> 90% immunoblasts
+*Trapezoid shaped large lymphoid cells with significant [[basophilic]] cytoplasm
+'''Anaplastic'''
+*Very large cells with a round, oval, or polygonal shape that may resemble Reed-Sternberg cells
+| align="center" style="background:#F5F5F5;" + |_
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Mantle cell lymphoma]]<br><ref>{{Cite journal
+ | author = [[Itziar Salaverria]], [[Cristina Royo]], [[Alejandra Carvajal-Cuenca]], [[Guillem Clot]], [[Alba Navarro]], [[Alejandra Valera]], [[Joo Y. Song]], [[Renata Woroniecka]], [[Grzegorz Rymkiewicz]], [[Wolfram Klapper]], [[Elena M. Hartmann]], [[Pierre Sujobert]], [[Iwona Wlodarska]], [[Judith A. Ferry]], [[Philippe Gaulard]], [[German Ott]], [[Andreas Rosenwald]], [[Armando Lopez-Guillermo]], [[Leticia Quintanilla-Martinez]], [[Nancy L. Harris]], [[Elaine S. Jaffe]], [[Reiner Siebert]], [[Elias Campo]] & [[Silvia Bea]]
+ | title = CCND2 rearrangements are the most frequent genetic events in cyclin D1(-) mantle cell lymphoma
+ | journal = [[Blood]]
+ | volume = 121
+ | issue = 8
+ | pages = 1394–1402
+ | year = 2013
+ | month = February
+ | doi = 10.1182/blood-2012-08-452284
+ | pmid = 23255553
+}}</ref><ref>{{Cite journal
+ | author = [[Markus Tiemann]], [[Carsten Schrader]], [[Wolfram Klapper]], [[Martin H. Dreyling]], [[Elias Campo]], [[Andrew Norton]], [[Francoise Berger]], [[Philip Kluin]], [[German Ott]], [[Stephano Pileri]], [[Ennio Pedrinis]], [[Alfred C. Feller]], [[Hartmut Merz]], [[Dirk Janssen]], [[Martin L. Hansmann]], [[Han Krieken]], [[Peter Moller]], [[Harald Stein]], [[Michael Unterhalt]], [[Wolfgang Hiddemann]] & [[Reza Parwaresch]]
+ | title = Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network
+ | journal = [[British journal of haematology]]
+ | volume = 131
+ | issue = 1
+ | pages = 29–38
+ | year = 2005
+ | month = October
+ | doi = 10.1111/j.1365-2141.2005.05716.x
+ | pmid = 16173960
+}}</ref><ref>{{Cite journal
+ | author = [[L. H. Argatoff]], [[J. M. Connors]], [[R. J. Klasa]], [[D. E. Horsman]] & [[R. D. Gascoyne]]
+ | title = Mantle cell lymphoma: a clinicopathologic study of 80 cases
+ | journal = [[Blood]]
+ | volume = 89
+ | issue = 6
+ | pages = 2067–2078
+ | year = 1997
+ | month = March
+ | pmid = 9058729
+}}</ref><ref>{{Cite journal
+ | author = [[Markus Tiemann]], [[Carsten Schrader]], [[Wolfram Klapper]], [[Martin H. Dreyling]], [[Elias Campo]], [[Andrew Norton]], [[Francoise Berger]], [[Philip Kluin]], [[German Ott]], [[Stephano Pileri]], [[Ennio Pedrinis]], [[Alfred C. Feller]], [[Hartmut Merz]], [[Dirk Janssen]], [[Martin L. Hansmann]], [[Han Krieken]], [[Peter Moller]], [[Harald Stein]], [[Michael Unterhalt]], [[Wolfgang Hiddemann]] & [[Reza Parwaresch]]
+ | title = Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network
+ | journal = [[British journal of haematology]]
+ | volume = 131
+ | issue = 1
+ | pages = 29–38
+ | year = 2005
+ | month = October
+ | doi = 10.1111/j.1365-2141.2005.05716.x
+ | pmid = 16173960
+}}</ref><ref>{{Cite journal
+ | author = [[Julie M. Vose]]
+ | title = Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management
+ | journal = [[American journal of hematology]]
+ | volume = 92
+ | issue = 8
+ | pages = 806–813
+ | year = 2017
+ | month = August
+ | doi = 10.1002/ajh.24797
+ | pmid = 28699667
+}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell lymphoma
+* [[CD5 (protein)|CD5]] positive antigen in pre germinal center of B-cell
+* [[Chromosomal translocation]] at t(11:14)
+| align="left" style="background:#F5F5F5;" + |
+* [[Fatigue]]
+* [[Night sweats]]
+* [[Weight Loss]]
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="left" style="background:#F5F5F5;" + |
+* Palpable masses in [[skin]], [[breast]], and [[salivary glands]]
+* [[Generalized lymphadenopathy]]
+| align="left" style="background:#F5F5F5;" + |
+* Extranodal involvement of [[GI tract]], [[lungs]], and [[CNS]]
+* [[X linked mental retardation-precocious puberty-obesity|Mental retardation]]
+| align="left" style="background:#F5F5F5;" + |
+* CD5<sup>+</sup>
+* B-cell antigen positive
+* Overexpression of Cyclin D1
+| align="left" style="background:#F5F5F5;" + |
+* Germinal centers filled by small-to-medium atypical [[lymphocytes]]
+* Nodular appearance
+| align="center" style="background:#F5F5F5;" + |[[Abdominal distention]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |B-lymphoblastic leukemia/lymphoma<br><ref name="pmid11075849">{{cite journal |vauthors=Lin P, Jones D, Dorfman DM, Medeiros LJ |title=Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement |journal=Am. J. Surg. Pathol. |volume=24 |issue=11 |pages=1480–90 |date=November 2000 |pmid=11075849 |doi= |url=}}</ref><ref name="pmid30410844">{{cite journal |vauthors=Javed Z, Hanif F |title=A Rare Presentation of Precursor B-cell Lymphoblastic Lymphoma in a Child |journal=Cureus |volume=10 |issue=8 |pages=e3238 |date=August 2018 |pmid=30410844 |pmc=6209516 |doi=10.7759/cureus.3238 |url=}}</ref><ref name="pmid25548762">{{cite journal |vauthors=Kim JY, Om SY, Shin SJ, Kim JE, Yoon DH, Suh C |title=Case series of precursor B-cell lymphoblastic lymphoma |journal=Blood Res |volume=49 |issue=4 |pages=270–4 |date=December 2014 |pmid=25548762 |pmc=4278010 |doi=10.5045/br.2014.49.4.270 |url=}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Precursor B-cell lymphoma
+*Unknown
+*Inherited mutations of [[PAX5]], [[ETV6]], and [[TP53BP2|TP53]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+* Painless mass at extranodal sites mainly of the [[skin]], [[bone]] and [[lymph node]].
+| align="left" style="background:#F5F5F5;" + |
+* [[Bleeding]]
+*[[Dyspnea]]
+*[[Joint pain]]
+| align="left" style="background:#F5F5F5;" + |
+* CD20+
+* CD10+
+| align="left" style="background:#F5F5F5;" + |
+* Consists of small to medium-sized lymphoid cells with blastic nuclear chromatin and a high mitotic rate.
+| align="left" style="background:#F5F5F5;" + |
+* [[Down syndrome]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Follicular lymphoma]]<br><ref name="pmid25176983">{{cite journal| author=Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES| title=Early lymphoid lesions: conceptual, diagnostic and clinical challenges. | journal=Haematologica | year= 2014 | volume= 99 | issue= 9 | pages= 1421-32 | pmid=25176983 | doi=10.3324/haematol.2014.107938 | pmc=4562530 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25176983  }}</ref><ref name="pmid11877266">{{cite journal| author=Lorsbach RB, Shay-Seymore D, Moore J, Banks PM, Hasserjian RP, Sandlund JT et al.| title=Clinicopathologic analysis of follicular lymphoma occurring in children. | journal=Blood | year= 2002 | volume= 99 | issue= 6 | pages= 1959-64 | pmid=11877266 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11877266  }}</ref><ref>[http://pleiad.umdnj.edu/hemepath/follicular/follicular.html Overview] at [[University of Medicine and Dentistry of New Jersey|UMDNJ]]</ref><ref name="pmid16075463">{{cite journal |author=Bosga-Bouwer AG, Haralambieva E, Booman M, ''et al.'' |title=BCL6 alternative translocation breakpoint cluster region associated with follicular lymphoma grade 3B |journal=Genes Chromosomes Cancer |volume=44 |issue=3 |pages=301–4 |date=November 2005 |pmid=16075463 |doi=10.1002/gcc.20246}}</ref><ref name="pmid7028244">{{cite journal| author=Winberg CD, Nathwani BN, Bearman RM, Rappaport H| title=Follicular (nodular) lymphoma during the first two decades of life: a clinicopathologic study of 12 patients. | journal=Cancer | year= 1981 | volume= 48 | issue= 10 | pages= 2223-35 | pmid=7028244 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7028244  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell lymphoma
+* Reciprocal translocation t(14;18)
+* Overexpression of ''BCL2''
+| align="left" style="background:#F5F5F5;" + |20% of patients present with:
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |±
+| align="left" style="background:#F5F5F5;" + |
+* Painless peripheral [[adenopathy]] in the [[cervical]], [[axillary]], [[inguinal]], and/or femoral regions
+* Asymptomatic large abdominal mass
+| align="left" style="background:#F5F5F5;" + |
+* [[Seizures]]
+*[[Chest pain]]
+*[[Bone pain]]
+*[[Cough]]
+*[[Dyspnea]]
+| align="left" style="background:#F5F5F5;" + |
+* CD21
+* CD23
+| align="left" style="background:#F5F5F5;" + |
+* Nodular growth pattern
+| align="left" style="background:#F5F5F5;" + |
+* Most common clinically indolent NHL
+* Peripheral nerve compression
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Burkitt's lymphoma]]<br><ref>Burkitt's Lymphoma. Wikibooks. https://en.wikibooks.org/wiki/Radiation_Oncology/NHL/Burkitt_lymphoma#Pathology Accessed on October,5 2015</ref><ref name="pmid12610094">{{cite journal |author=Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L |title=Burkitt's lymphoma: new insights into molecular pathogenesis |journal=J. Clin. Pathol. |volume=56 |issue=3 |pages=188–92 |year=2003 |month=March |pmid=12610094 |pmc=1769902 |doi= |url=http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=12610094}}</ref><ref name="ChuangYe2007">{{cite journal|last1=Chuang|first1=Shih-Sung|last2=Ye|first2=Hongtao|last3=Du|first3=Ming-Qing|last4=Lu|first4=Chin-Li|last5=Dogan|first5=Ahmet|last6=Hsieh|first6=Pin-Pen|last7=Huang|first7=Wan-Ting|last8=Jung|first8=Yun-Chih|title=Histopathology and Immunohistochemistry in Distinguishing Burkitt Lymphoma From Diffuse Large B-Cell Lymphoma With Very High Proliferation Index and With or Without a Starry-Sky Pattern|journal=American Journal of Clinical Pathology|volume=128|issue=4|year=2007|pages=558–564|issn=0002-9173|doi=10.1309/EQJR3D3V0CCQGP04}}</ref><ref name="pmid977186">{{cite journal| author=Magrath IT, Simon RM| title=Immunosuppression in Burkitt's lymphoma. II. Peripheral blood lymphocyte populations related to clinical status. | journal=Int J Cancer | year= 1976 | volume= 18 | issue= 4 | pages= 399-408 | pmid=977186 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=977186  }}</ref><ref name="pmid25856946">{{cite journal| author=Basavaraj A, Shinde A, Kulkarni R, Kadam DB, Chugh A| title=HIV associated Burkitt's lymphoma. | journal=J Assoc Physicians India | year= 2014 | volume= 62 | issue= 8 | pages= 723-7 | pmid=25856946 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25856946  }}</ref><ref name="RoweFitzsimmons2014">{{cite journal|last1=Rowe|first1=Martin|last2=Fitzsimmons|first2=Leah|last3=Bell|first3=Andrew I|title=Epstein-Barr virus and Burkitt lymphoma|journal=Chinese Journal of Cancer|year=2014|issn=1000467X|doi=10.5732/cjc.014.10190}}</ref><ref name="pmid30290813">{{cite journal| author=Ekemen S, Uzay A, Bassullu N, Dikicioglu-Cetin E, Matsuda K, Ince U et al.| title=Does it take three to tango? An unsuspected multimorbidity of CD8+ T cell lymphoproliferative disorder, malaria, and EBV infection. | journal=Malar J | year= 2018 | volume= 17 | issue= 1 | pages= 349 | pmid=30290813 | doi=10.1186/s12936-018-2497-9 | pmc=6173833 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30290813  }}</ref><ref name="pmid26437030">{{cite journal| author=Kretzmer H, Bernhart SH, Wang W, Haake A, Weniger MA, Bergmann AK et al.| title=DNA methylome analysis in Burkitt and follicular lymphomas identifies differentially methylated regions linked to somatic mutation and transcriptional control. | journal=Nat Genet | year= 2015 | volume= 47 | issue= 11 | pages= 1316-1325 | pmid=26437030 | doi=10.1038/ng.3413 | pmc=5444523 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26437030  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell lymphoma
+* Translocation of chromosome 8 ''[[myc]]'' locus such as:
+** t(8;14)
+** t(2;8)
+** t(8;22)
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Night sweats]]
+* Unexplained [[weight loss]]
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+* [[Swollen lymph nodes]] in the neck, axilla, or groin
+* Jaw mass
+* [[Abdominal mass|Abdominal masses]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Ascites]]
+* [[Proptosis]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD19]]
+* [[CD20]]
+* [[CD22]]
+* [[CD10]]
+* BCL6
+* BCL2 and TdT
+| align="left" style="background:#F5F5F5;" + |
+* Round and small nucleoli
+* [[basophilic]] cytoplasm
+* "Starry-sky pattern"
+| align="left" style="background:#F5F5F5;" + |
+*[[Mononucleosis|EBV infection]]
+*[[HIV AIDS|HIV infection]]
+*[[Immunosuppression]]
+*[[Abdominal distension|Abdominal distention]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |B cell chronic lymphocytic leukemia/small lymphocytic lymphoma<br><ref name="KleinTu2001">{{cite journal|last1=Klein|first1=Ulf|last2=Tu|first2=Yuhai|last3=Stolovitzky|first3=Gustavo A.|last4=Mattioli|first4=Michela|last5=Cattoretti|first5=Giorgio|last6=Husson|first6=Hervé|last7=Freedman|first7=Arnold|last8=Inghirami|first8=Giorgio|last9=Cro|first9=Lilla|last10=Baldini|first10=Luca|last11=Neri|first11=Antonino|last12=Califano|first12=Andrea|last13=Dalla-Favera|first13=Riccardo|title=Gene Expression Profiling of B Cell Chronic Lymphocytic Leukemia Reveals a Homogeneous Phenotype Related to Memory B Cells|journal=The Journal of Experimental Medicine|volume=194|issue=11|year=2001|pages=1625–1638|issn=0022-1007|doi=10.1084/jem.194.11.1625}}</ref>
-==[[Hepatosplenic T cell lymphoma overview|Overview]]==
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell Lymphoma
+* Ch 13 abnormalties
+*
+| align="left" style="background:#F5F5F5;" + |
+% of patients present with:
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+* Painless [[lymphadenopathy]]
+| align="left" style="background:#F5F5F5;" + |
+* May be asymptomatic
+* [[Fatigue]]
+* Recurrent infections
+* [[Hepatosplenomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* CD5
+* CD38
+| align="left" style="background:#F5F5F5;" + |
+* Monoclonal small well differentiated B cells.
+* Significant  number of smudge cells or basket cells.
+| align="left" style="background:#F5F5F5;" + |
+* [[Autoimmune hemolytic anemia]]
+* Red cell aplasia
+* Autoimmune [[thrombocytopenia]]
+|-
+! rowspan="3" align="center" style="background:#DCDCDC;" + |Marginal zone lymphoma
+! align="center" style="background:#DCDCDC;" + |Extranodal marginal zone B-cell lymphoma of [[MALT lymphoma|mucosa-associated lymphoid tissue]] (MALT) type<br><ref name="sympmalylymmnoingastry">Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref><ref name="riskfactorsmaltlymphoma1">Risks of Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 25, 2016</ref><ref name="KinkadeEsan2015">{{cite journal|last1=Kinkade|first1=Zoe|last2=Esan|first2=Olukemi A.|last3=Rosado|first3=Flavia G.|last4=Craig|first4=Michael|last5=Vos|first5=Jeffrey A.|title=Ileal mucosa-associated lymphoid tissue lymphoma presenting with small bowel obstruction: a case report|journal=Diagnostic Pathology|volume=10|issue=1|year=2015|issn=1746-1596|doi=10.1186/s13000-015-0353-6}}</ref><ref name="sympromaltlymphoma2">Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref><ref name="sympromaltlymphoma22">Symptoms of MALT lymphoma. Cancer research UK 2016. http://www.cancerresearchuk.org/about-cancer/type/non-hodgkins-lymphoma/about/types/mucosaassociated-lymphoid-tissue-lymphoma. Accessed on January 28, 2016</ref><ref name="sympromaltlymphoma1">Signs and symptoms of gastric lymphoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Gastric_lymphoma. Accessed on January 28, 2016</ref><ref name="nongastricorbitlymphomasym1">Clinical presentation of orbital lymphoma. Dr Craig Hacking and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/orbital-lymphoma. Accessed on January 28, 2016</ref><ref name="sympmalylymmnoingastry2">Non-gastric lymphomas – causes, symptoms and treatments. Lymphoma association 2016. https://www.lymphomas.org.uk/sites/default/files/pdfs/Non-Gastric-malt-lymphoma.pdf. Accessed on January 28, 2016</ref><ref>{{cite journal|last1=Taal|first1=B G|last2=Boot|first2=H|last3=van Heerde|first3=P|last4=de Jong|first4=D|last5=Hart|first5=A A|last6=Burgers|first6=J M|title=Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept.|journal=Gut|date=1 October 1996|volume=39|issue=4|pages=556–561|doi=10.1136/gut.39.4.556}}</ref><ref name="pmid16950858">{{cite journal| author=Bacon CM, Du MQ, Dogan A| title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 4 | pages= 361-72 | pmid=16950858 | doi=10.1136/jcp.2005.031146 | pmc=PMC2001121 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16950858  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell lymphoma
+* Recurrent translocation of such as:
+** t(1;14)(p22;q32)
+** t(11;18)(q21;q21)
+** t(14;18)(q32;q21)
+** t(3;14)(p14.1;q32)
+| align="left" style="background:#F5F5F5;" + |
+*B symptoms may or may not be present
+| align="center" style="background:#F5F5F5;" + |±
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="left" style="background:#F5F5F5;" + |
+*Painless or painfull [[lymphadenopathy]] which can be gastric or non-gastric.
+*[[Orbital mass causes|Lump in the superior lateral quadrant of the orbit]], [[breast]], [[neck]], or [[salivary gland]]
+| align="left" style="background:#F5F5F5;" + |
+*Symptoms depend on the location of the tumor:
+*[[Fatigue]]
+*[[Heartburn]]
+*[[Fever]]
+*[[Cough]] with [[Hemoptysis|blood in sputum]],
+*[[Shortness of breath]]
+| align="left" style="background:#F5F5F5;" + |B-cell associated antigens that co-express
+* [[BCL-2]]
-==[[Hepatosplenic T cell lymphoma historical perspective|Historical Perspective]]==
+*[[CD19]]
+*[[CD20]]
+*[[CD22]]
+*[[CD79a]]
+Negative for:
+*[[CD5]]
+*[[CD10]]
+*[[CD43]]
+*[[cyclin D1]].
+| align="left" style="background:#F5F5F5;" + |
+*Presence of dense diffuse lymphoid infiltrate of marginal‐zone cells in lamina propria
+*Prominent lymphoepithelial lesions and consisting of small atypical cells with monocytoid features.
+| align="left" style="background:#F5F5F5;" + |
+*''[[Helicobacter|H. pylori]]'' infection
+*[[Hashimoto's thyroiditis]]
+*[[Sjogren’s syndrome]]
+*[[Celiac disease]].
+|-
+! align="center" style="background:#DCDCDC;" + |[[Splenic marginal zone lymphoma]]<br><ref name="pmid11337382">{{cite journal |vauthors=Hernández JM, García JL, Gutiérrez NC, Mollejo M, Martínez-Climent JA, Flores T, González MB, Piris MA, San Miguel JF |title=Novel genomic imbalances in B-cell splenic marginal zone lymphomas revealed by comparative genomic hybridization and cytogenetics |journal=Am. J. Pathol. |volume=158 |issue=5 |pages=1843–50 |date=May 2001 |pmid=11337382 |pmc=1891967 |doi=10.1016/S0002-9440(10)64140-5 |url=}}</ref><ref name="pmid15642391">{{cite journal |vauthors=Andersen CL, Gruszka-Westwood A, Atkinson S, Matutes E, Catovsky D, Pedersen RK, Pedersen BB, Pulczynski S, Hokland P, Jacobsen E, Koch J |title=Recurrent genomic imbalances in B-cell splenic marginal-zone lymphoma revealed by comparative genomic hybridization |journal=Cancer Genet. Cytogenet. |volume=156 |issue=2 |pages=122–8 |date=January 2005 |pmid=15642391 |doi=10.1016/j.cancergencyto.2004.04.026 |url=}}</ref><ref name="pmid20479288">{{cite journal |vauthors=Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F |title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group |journal=Blood |volume=116 |issue=9 |pages=1479–88 |date=September 2010 |pmid=20479288 |doi=10.1182/blood-2010-02-267476 |url=}}</ref><ref>Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on December 22, 2015</ref><ref name="pmid12916862">{{cite journal |vauthors=Weng WK, Levy S |title=Hepatitis C virus (HCV) and lymphomagenesis |journal=Leuk. Lymphoma |volume=44 |issue=7 |pages=1113–20 |date=July 2003 |pmid=12916862 |doi=10.1080/1042819031000076972 |url=}}</ref><ref name="pmid11739181">{{cite journal |vauthors=Quinn ER, Chan CH, Hadlock KG, Foung SK, Flint M, Levy S |title=The B-cell receptor of a hepatitis C virus (HCV)-associated non-Hodgkin lymphoma binds the viral E2 envelope protein, implicating HCV in lymphomagenesis |journal=Blood |volume=98 |issue=13 |pages=3745–9 |date=December 2001 |pmid=11739181 |doi= |url=}}</ref><ref name="pmid20530156">{{cite journal |vauthors=Chuang SS, Liao YL, Chang ST, Hsieh YC, Kuo SY, Lu CL, Hwang WS, Lin IH, Tsao CJ, Huang WT |title=Hepatitis C virus infection is significantly associated with malignant lymphoma in Taiwan, particularly with nodal and splenic marginal zone lymphomas |journal=J. Clin. Pathol. |volume=63 |issue=7 |pages=595–8 |date=July 2010 |pmid=20530156 |doi=10.1136/jcp.2010.076810 |url=}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell lymphoma
+* Clonal rearrangements of the [[immunoglobulin]] genes (heavy and light chains)
+** Deletion 7q21-32
+** Translocations of the CDK6 gene located on 7q21
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+*Painless swelling in the neck, [[axilla]], [[groin]], [[thorax]], and [[abdomen]]
+| align="left" style="background:#F5F5F5;" + |
+*Chest pain
+*[[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |
+*[[CD20]]
+*[[CD79a]]
+| align="left" style="background:#F5F5F5;" + |
+*Small [[lymphocytes]]
+*Transformed blasts
+*Epithelial [[histocytes]]
+*Plasmacytic differentiation of neoplastic cells
+| align="left" style="background:#F5F5F5;" + |
+*[[Hepatitis C]] infection
+|-
+! align="center" style="background:#DCDCDC;" + |[[Nodal marginal zone B-cell lymphoma]]<br><ref name="SpinaKhiabanian2016">{{cite journal|last1=Spina|first1=V.|last2=Khiabanian|first2=H.|last3=Messina|first3=M.|last4=Monti|first4=S.|last5=Cascione|first5=L.|last6=Bruscaggin|first6=A.|last7=Spaccarotella|first7=E.|last8=Holmes|first8=A. B.|last9=Arcaini|first9=L.|last10=Lucioni|first10=M.|last11=Tabbo|first11=F.|last12=Zairis|first12=S.|last13=Diop|first13=F.|last14=Cerri|first14=M.|last15=Chiaretti|first15=S.|last16=Marasca|first16=R.|last17=Ponzoni|first17=M.|last18=Deaglio|first18=S.|last19=Ramponi|first19=A.|last20=Tiacci|first20=E.|last21=Pasqualucci|first21=L.|last22=Paulli|first22=M.|last23=Falini|first23=B.|last24=Inghirami|first24=G.|last25=Bertoni|first25=F.|last26=Foa|first26=R.|last27=Rabadan|first27=R.|last28=Gaidano|first28=G.|last29=Rossi|first29=D.|title=The genetics of nodal marginal zone lymphoma|journal=Blood|volume=128|issue=10|year=2016|pages=1362–1373|issn=0006-4971|doi=10.1182/blood-2016-02-696757}}</ref><ref name="canadiancancer">Nodal marginal zone lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/nodal-marginal-zone-lymphoma/?region=nb Accessed on March 4, 2016</ref>
+| align="left" style="background:#F5F5F5;" + |
+*Mature B-cell Lymphoma
+*Stimulation of antigen receptor by autoantigen and co-stimulatory molecule CD40
+*[[Mutation|Mutations]] in [[KMT2D]], PTTPRD, [[NOTCH2]], [[KLF2]]
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+*Painless [[swelling]] in the neck, [[axilla]], [[groin]], [[thorax]], and [[abdomen]]
+*Generalized and local [[lymphadenopathy]]
+| align="left" style="background:#F5F5F5;" + |
+*[[Hemorrhage]]
+*[[Dyspepsia]]
+| align="left" style="background:#F5F5F5;" + |
+*Follicular cells in reactive zone
+*Centrocyte like cells in marginal zone lymphoma
+| align="left" style="background:#F5F5F5;" + |
+*Centrocytes
+*Centroblasts
+*[[Plasma cell|Plasma cells]]
+*[[Immunoblast|Immunoblasts]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Hepatitis C]] infection
-==[[Hepatosplenic T cell lymphoma classification|Classification]]==
+* Chronic infectious conditions or autoimmune processes, such as:
+**[[Gastritis|''H pylori'' gastritis]]
+**[[Hashimoto thyroiditis]]
+**[[Sjögren's syndrome|Sjögren syndrome]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Waldenström's macroglobulinemia|Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)]]<br><ref>{{Cite journal
+ | author = [[Steven P. Treon]], [[Lian Xu]], [[Guang Yang]], [[Yangsheng Zhou]], [[Xia Liu]], [[Yang Cao]], [[Patricia Sheehy]], [[Robert J. Manning]], [[Christopher J. Patterson]], [[Christina Tripsas]], [[Luca Arcaini]], [[Geraldine S. Pinkus]], [[Scott J. Rodig]], [[Aliyah R. Sohani]], [[Nancy Lee Harris]], [[Jason M. Laramie]], [[Donald A. Skifter]], [[Stephen E. Lincoln]] & [[Zachary R. Hunter]]
+ | title = MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia
+ | journal = [[The New England journal of medicine]]
+ | volume = 367
+ | issue = 9
+ | pages = 826–833
+ | year = 2012
+ | month = August
+ | doi = 10.1056/NEJMoa1200710
+ | pmid = 22931316
+}}</ref><ref name="AC">{{cite journal |vauthors=Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT |title=Renal MALT lymphoma associated with Waldenström macroglobulinemia |journal=J. Formos. Med. Assoc. |volume=113 |issue=4 |pages=255–7 |year=2014 |pmid=24685302 |doi=10.1016/j.jfma.2011.02.007 |url=}}</ref><ref name="AC2">{{cite journal |vauthors=Chi PJ, Pei SN, Huang TL, Huang SC, Ng HY, Lee CT |title=Renal MALT lymphoma associated with Waldenström macroglobulinemia |journal=J. Formos. Med. Assoc. |volume=113 |issue=4 |pages=255–7 |year=2014 |pmid=24685302 |doi=10.1016/j.jfma.2011.02.007 |url=}}</ref><ref name="pmid11736938">{{cite journal| author=García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A et al.| title=Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. | journal=Br J Haematol | year= 2001 | volume= 115 | issue= 3 | pages= 575-82 | pmid=11736938 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11736938  }}</ref><ref name="pmid12720138">{{cite journal| author=Merlini G, Baldini L, Broglia C, Comelli M, Goldaniga M, Palladini G et al.| title=Prognostic factors in symptomatic Waldenstrom's macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 211-5 | pmid=12720138 | doi=10.1053/sonc.2003.50064 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720138  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature B-cell Lymphoma
+* Mutation of the [[MYD88|MYD88 gene]] (L265P)
+* [[Nonsense mutation|Nonsense]] or [[Frameshift mutation|frameshift mutations]] in the CXR4 gene
+| align="left" style="background:#F5F5F5;" + |
+*[[Weakness]]
+*[[Anorexia]]
+*[[Weight loss]]
+*[[Fever]]
+*
+| align="center" style="background:#F5F5F5;" + | –
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |
+* Generalized and local [[lymphadenopathy]]
+| align="left" style="background:#F5F5F5;" + |
+*Retinal [[hemorrhage]] with [[Blurred vision|blurring of vision]]
+*[[Vertigo]]
+*[[Dizziness]]
+*[[Headache]]
+*[[Nystagmus]]
+*[[Tinnitus]]
+*[[Ataxia]]
+*[[Bleeding]]
+| align="left" style="background:#F5F5F5;" + |Express pan B-cell antigens
+* [[CD19]]
+* [[CD20]]
+* [[CD22]]
+* [[CD79A]]
+* [[CD5]]
+Variable expression of
+* [[CD11c]]
+* [[CD43]],
+* [[CD25]]
+Majority express:
+* [[IgM]] surface immunoglobulin
+Fewer express
+* [[IgG]] or [[Immunoglobulin A|IgA]]
+* Lack [[Immunoglobulin D|IgD]]
+| align="left" style="background:#F5F5F5;" + |
+* Russell bodies (intracytoplasmic inclusions) and Dutcher bodies (intranuclear inclusions)
+* Prominent epithelioid histiocytes
+| align="left" style="background:#F5F5F5;" + |
+* [[Diffuse large B-cell lymphoma]]
+* [[Myelodysplastic syndrome]]/[[Acute myeloid leukemia]]
+* [[Brain tumor]]
+* [[MALT lymphoma|Renal MALT lymphoma]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Hairy cell leukemia]]<br><ref name="pmid16990106">{{cite journal |vauthors=Matutes E |title=Immunophenotyping and differential diagnosis of hairy cell leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=20 |issue=5 |pages=1051–63 |date=October 2006 |pmid=16990106 |doi=10.1016/j.hoc.2006.06.012 |url=}}</ref><ref name="pmid169901062">{{cite journal |vauthors=Matutes E |title=Immunophenotyping and differential diagnosis of hairy cell leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=20 |issue=5 |pages=1051–63 |date=October 2006 |pmid=16990106 |doi=10.1016/j.hoc.2006.06.012 |url=}}</ref><ref name="pmid22210875">{{cite journal |vauthors=Xi L, Arons E, Navarro W, Calvo KR, Stetler-Stevenson M, Raffeld M, Kreitman RJ |title=Both variant and IGHV4-34-expressing hairy cell leukemia lack the BRAF V600E mutation |journal=Blood |volume=119 |issue=14 |pages=3330–2 |date=April 2012 |pmid=22210875 |pmc=3321859 |doi=10.1182/blood-2011-09-379339 |url=}}</ref><ref name="pmid21910720">{{cite journal |vauthors=Boyd EM, Bench AJ, van 't Veer MB, Wright P, Bloxham DM, Follows GA, Scott MA |title=High resolution melting analysis for detection of BRAF exon 15 mutations in hairy cell leukaemia and other lymphoid malignancies |journal=Br. J. Haematol. |volume=155 |issue=5 |pages=609–12 |date=December 2011 |pmid=21910720 |doi=10.1111/j.1365-2141.2011.08868.x |url=}}</ref><ref name="pmid15710587">{{cite journal |vauthors=Forconi F, Raspadori D, Lenoci M, Lauria F |title=Absence of surface CD27 distinguishes hairy cell leukemia from other leukemic B-cell malignancies |journal=Haematologica |volume=90 |issue=2 |pages=266–8 |date=February 2005 |pmid=15710587 |doi= |url=}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Production of [[Cytokine|cytokines]], such as [[TNF alpha]] and IL-2R, provide important stimuli for [[malignant]] [[B cell|B cells]] proliferation in hairy cell leukemia.
+** The ''p38-MAPK-JNK'' cascade
+** The ''MEK-ERK'' cascade
+** The ''Phosphatidylinositol 3 kinase (PI3K)-AKT'' cascade
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Night sweat|Night sweats]]
+*[[Fatigue]]
+*Easy [[bruising]] or [[bleeding]]
+*Generalized weakness
+*[[Weight loss]]
+*Recurrent [[Infection|infections]]
+*Early satiety
+| align="center" style="background:#F5F5F5;" + |–
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + |–
+| align="left" style="background:#F5F5F5;" + |
+*[[Pallor]]
+*[[Petechiae]]
+*[[Splenomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* N/A
+| align="left" style="background:#F5F5F5;" + |
+*[[Annexin A1]]
+*[[CD20]]
+*[[CD25]]
+*[[CD103]]
+*[[CD19]]
+*[[CD11c]]
+*[[FMC7]]
+| align="left" style="background:#F5F5F5;" + |
+*Small cells with "fried egg"-like appearance
+*Well-demarcated thread-like [[cytoplasmic]] extensions
+*Clear cytoplasm
+*Central round [[nucleus]]
+*Peri-nuclear clearing ("water-clear rim" appearance)
+| align="left" style="background:#F5F5F5;" + |
+* [[Abdominal distension|Abdominal distention]]
+|-
+! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Constitutional symptoms
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Rash
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Abdominal pain
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Diarrhea
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mass/Lesion
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Other
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunochemistry
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathology
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
+|-
+! colspan="12" align="center" style="background:#7d7d7d; color: #FFFFFF;" + |Non-Hodgkin's Lymphoma (T Cell Lymphoma)
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |Precursor T-cell lymphoblastic leukemia/lymphoma<br><ref name="pmid28892922">{{cite journal| author=Shelly D, Gujral S| title=Early T-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma: Immunohistochemical Evaluation of Four Lymph Node Biopsies. | journal=J Clin Diagn Res | year= 2017 | volume= 11 | issue= 7 | pages= EL01-EL02 | pmid=28892922 | doi=10.7860/JCDR/2017/29352.10164 | pmc=5583929 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28892922  }}</ref><ref name="pmid26276771">{{cite journal| author=You MJ, Medeiros LJ, Hsi ED| title=T-lymphoblastic leukemia/lymphoma. | journal=Am J Clin Pathol | year= 2015 | volume= 144 | issue= 3 | pages= 411-22 | pmid=26276771 | doi=10.1309/AJCPMF03LVSBLHPJ | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276771  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Precursor T-cell lymphomas
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Fatigue]]
+* [[Weight loss]]
+* [[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* In most patients the [[mediastinal mass]] is anterior; associated with [[Pleural effusion|pleural effusions]]
+* Can produce such complications as [[superior vena cava syndrome]]
+* Skin and bone abnormalities
+* [[Hepatosplenomegaly|Enlarged liver and/or spleen]]
+*[[Tracheal compression|Tracheal obstruction]]
+*[[Pericardial effusion|Pericardial effusions]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Skin rash]]
+* [[Chest pain]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD3 (immunology)|CD3]]
+* [[CD7]]
+* TDT
+| align="left" style="background:#F5F5F5;" + |
+* Blast-like cells with scant [[cytoplasm]], convoluted [[Cell nucleus|nuclei]], fine [[chromatin]], indistinct [[Nucleolus|nucleoli]].
+* Some [[Lymphoblast|lymphoblasts]] have cytoplasmic [[Pseudopod|pseudopods]]
+| align="left" style="background:#F5F5F5;" + |
+* Males in their teens to early twenties
+* [[Lymphadenopathy CT scan|Lymphadenopathy]] in cervical, [[Supraclavicular lymph nodes|supraclavicular]],  [[Axillary lymph nodes|axillary]] ,and [[mediastinum]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell granular lymphocytic<br><ref name="Rose2004">{{cite journal|last1=Rose|first1=M. G.|title=T-Cell Large Granular Lymphocyte Leukemia and Related Disorders|journal=The Oncologist|volume=9|issue=3|year=2004|pages=247–258|issn=1083-7159|doi=10.1634/theoncologist.9-3-247}}</ref><ref name="pmid22800517">{{cite journal| author=Liu EB, Chen HS, Zhang PH, Li ZQ, Sun Q, Yang QY et al.| title=[Hematopathologic features of T-cell large granular lymphocytic leukemia]. | journal=Zhonghua Bing Li Xue Za Zhi | year= 2012 | volume= 41 | issue= 4 | pages= 229-33 | pmid=22800517 | doi=10.3760/cma.j.issn.0529-5807.2012.04.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22800517  }}</ref><ref name="pmid15958859">{{cite journal| author=Osuji N, Matutes E, Catovsky D, Lampert I, Wotherspoon A| title=Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 7 | pages= 935-41 | pmid=15958859 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15958859  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Clonal proliferation of [[CD8|CD8 T cells]]
+* Constant immune stimulation
+* Absence of homeostatic [[apoptosis]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Fatigue]]
+* [[Fever]]
+* [[Sleep hyperhidrosis|Night sweats]]
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* [[Lymphadenopathy]]
+* [[Hepatosplenomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* Frequent [[Infection|infections]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD8]]
+* [[CD57]]
+* [[T cell receptor|TCRαβ]]
+* [[CD3 (immunology)|CD3]]
+* [[CD16]]
+* [[Granzyme|Granzyme b]]
+* [[TIA1]]
+| align="left" style="background:#F5F5F5;" + |
+* Medium / large [[Lymphocyte|lymphocytes]] with abundant cytoplasm having azurophilic [[Granule (cell biology)|granules]]
+| align="left" style="background:#F5F5F5;" + |May be associated with the following conditions:
+* [[Monoclonal gammopathy of undetermined significance|Monoclonal gammopathy of unknown significance]] (MGUS)
+* [[Chronic lymphocytic leukemia|B-chronic lymphocytic leukemia]] (B-CLL)
+* [[Hairy cell leukemia]]
+* [[Myeloid|Myeloid hypoplasia]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell prolymphocytic leukemia<br><ref name="pmid23382603">{{cite journal| author=Graham RL, Cooper B, Krause JR| title=T-cell prolymphocytic leukemia. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 19-21 | pmid=23382603 | doi= | pmc=3523759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382603  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Abnormal proliferation of mature [[T cell|T cells]] (post thymic)
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Fatigue]]
+* [[Weight loss]]
+* [[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* [[Lymphadenopathy]]
+* [[Splenomegaly]]
+* [[Hepatomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Skin rash]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD2]]
+* [[CD3 (immunology)|CD3]]
+* [[CD4]]
+* [[CD5]]
+* [[CD7]]
+* CD56
+* [[TCL1A|TCL1]]
+| align="left" style="background:#F5F5F5;" + |
+*[[Lymphocytes]] with abundant [[basophilic]], nongranular cytoplasm, atypical nucleus
+*Cytoplasmic protrusions (blebs)
+| align="left" style="background:#F5F5F5;" + |
+*[[Pleural effusion]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Adult T-cell leukemia|Adult T cell leukemia/lymphoma]]<br><ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="pmid180426932">Matutes E (2007) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18042693 Adult T-cell leukaemia/lymphoma.] ''J Clin Pathol'' 60 (12):1373-7. [http://dx.doi.org/10.1136/jcp.2007.052456 DOI:10.1136/jcp.2007.052456] PMID: [http://pubmed.gov/18042693 18042693]</ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+* [[Human T-lymphotropic virus|Human T-lymphotrophic virus 1 infection]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Hepatosplenomegaly|Hepatosplenomagaly]]
+* T-cell deficiency leading [[immunodeficiency]] causing [[opportunistic infection]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* [[Hepatosplenomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Hypercalcemia]]
+* [[Skin lesion|Skin lesions]]
+* Lytic bone lesions
+* Elevated [[Lactate dehydrogenase|LDH]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Adult T-cell leukemia]] cells are typically positive for:
+* [[CD2]]
+* [[CD4]]
+* [[CD5]]
+* [[CD8]]
+| align="left" style="background:#F5F5F5;" + |
+* Charecterstic leukemia cells with sharp [[nuclear]] indentations and a prominent [[nucleoli]]
+* "Cloverleaf" or "flower" cells
+| align="left" style="background:#F5F5F5;" + |
+* [[Hypercalcemia]]
+|-
+! colspan="2" align="center" style="background:#DCDCDC;" + |[[Anaplastic large cell lymphoma]]<br><ref name="Al-AhmadMaertens2017">{{cite journal|last1=Al-Ahmad|first1=Selma|last2=Maertens|first2=Vincent|last3=Libeer|first3=Christophe|last4=Schelfhout|first4=Vera|last5=Vanhoenacker|first5=Filip|last6=Boeckx|first6=Nancy|last7=Vandevenne|first7=Marleen|title=The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature|journal=Acta Clinica Belgica|volume=72|issue=6|year=2017|pages=454–460|issn=1784-3286|doi=10.1080/17843286.2017.1312057}}</ref><ref name="pmid25197351">{{cite journal| author=Yu L, Yan LL, Yang SJ| title=Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 8 | pages= 4806-16 | pmid=25197351 | doi= | pmc=4152041 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25197351  }}</ref><ref name="pmid28652989">{{cite journal| author=de Campos FPF, Zerbini MCN, Felipe-Silva A, Simões AB, Lovisolo SM, da Fonseca LG et al.| title=Unusual clinical presentation of anaplastic large cell lymphoma. | journal=Autops Case Rep | year= 2014 | volume= 4 | issue= 1 | pages= 21-27 | pmid=28652989 | doi=10.4322/acr.2014.004 | pmc=5470561 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28652989  }}</ref><ref name="pmid17965727">{{cite journal |author=Watanabe M, Ogawa Y, Itoh K |title=Hypomethylation of CD30 CpG islands with aberrant JunB expression drives CD30 induction in Hodgkin lymphoma and anaplastic large cell lymphoma |journal=Lab. Invest. |volume=88 |issue=1 |pages=48–57 |date=January 2008 |pmid=17965727 |doi=10.1038/labinvest.3700696|display-authors=etal}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+Painless swelling in :
+* [[Neck masses|Neck]]
+* [[Axilla]]
+* [[Groin]]
+* [[Chest|Thorax]]
+* [[Abdominal mass|Abdomen]]
+| align="center" style="background:#F5F5F5;" + |
+* Skin rash
+* [[Chest pain]]
+* [[Abdominal pain]]
+* [[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |Strongly immunoreactive for :
+* [[CD30]]
+* Anaplastic lymphoma kinase (ALK)
+* EMA
+* [[vimentin]]
+| align="left" style="background:#F5F5F5;" + |
+*
+* Nucleoli tend to be more prominent
+* The cytoplasm may be either [[basophilic]] or [[eosinophilic]] and the cell might have many nuclei with dispersed or clumped [[chromatin]]
+| align="center" style="background:#F5F5F5;" + |
+* May be caused by textured breast implants
+|-
+! align="center" style="background:#DCDCDC;" + |Cutaneous T-cell lymphoma
+! align="center" style="background:#DCDCDC;" + |[[Mycosis fungoides]] / [[Sézary syndrome]]<br><ref name="FossGirardi2017">{{cite journal|last1=Foss|first1=Francine M.|last2=Girardi|first2=Michael|title=Mycosis Fungoides and Sezary Syndrome|journal=Hematology/Oncology Clinics of North America|volume=31|issue=2|year=2017|pages=297–315|issn=08898588|doi=10.1016/j.hoc.2016.11.008}}</ref><ref name="CampbellClark2010">{{cite journal|last1=Campbell|first1=J. J.|last2=Clark|first2=R. A.|last3=Watanabe|first3=R.|last4=Kupper|first4=T. S.|title=Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors|journal=Blood|volume=116|issue=5|year=2010|pages=767–771|issn=0006-4971|doi=10.1182/blood-2009-11-251926}}</ref><ref name="VonderheidBernengo2002">{{cite journal|last1=Vonderheid|first1=Eric C.|last2=Bernengo|first2=Maria Grazia|last3=Burg|first3=Günter|last4=Duvic|first4=Madeleine|last5=Heald|first5=Peter|last6=Laroche|first6=Liliane|last7=Olsen|first7=Elise|last8=Pittelkow|first8=Mark|last9=Russell-Jones|first9=Robin|last10=Takigawa|first10=Masahiro|last11=Willemze|first11=Rein|title=Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas|journal=Journal of the American Academy of Dermatology|volume=46|issue=1|year=2002|pages=95–106|issn=01909622|doi=10.1067/mjd.2002.118538}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell Lymphoma
+* The [[tumor]] [[Cell (biology)|cells]] originate from [[memory T cells]] or [[skin]] homing [[CD4+ T cells]] expressing [[cutaneous]] [[lymphocyte]] [[antigen]] (CLA) an<nowiki/>d [[chemokine]] [[receptors]] [[CCR4]]<nowiki/>and CCR7.
+* It is understood that cutaneous  T cell lymphoma (maycosis fungoides, Sezary sydrome ) is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.
+| align="left" style="background:#F5F5F5;" + |
+* [[Weight loss]]
+* [[Malaise]] and [[fatigue]]
+* [[Anemia]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* Cutaneous manifestations
+| align="left" style="background:#F5F5F5;" + |
+* [[Lymphadenopathy]]
+* May progress to [[Sezary syndrome]]
+| align="left" style="background:#F5F5F5;" + |
+* Beta F1+
+* [[CD2]]-/+
+* [[CD3]]+
+* [[CD3]]- ([[CD4]]-positive variant)
+* [[CD4]]+ ([[CD4]]-positive variant)
+* [[CD4]]-
+* [[CD5]]-
+* [[CD7]]+/-
+* [[CD8]]+
+* [[CD8]]- ([[CD4]]-positive variant),
+* [[Granzyme B|Granzyme B+]]
+* [[perforin]]+
+| align="left" style="background:#F5F5F5;" + |
+:* Polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical [[lymphoid]] cells
+:* These cells may line up individually along the epidermal basal layer
+:* The presence of spongiosis is highly suggestive of [[mycosis fungoides]]
-==[[Hepatosplenic T cell lymphoma pathophysiology|Pathophysiology]]==
+| align="center" style="background:#F5F5F5;" + |
+* [[Epidermis (skin)|Epidermal]] [[atrophy]] or poikiloderma
+* Generalized [[itching]]([[pruritus]])
+* [[Pain]] in the affected area of the skin.
+* [[Insomnia]]
+* Red ([[erythematous]]) patches scattered over the [[skin]] of the [[trunk]] and the [[extremities]]
+|-
+! rowspan="6" align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma
+! align="center" style="background:#DCDCDC;" + |[[Subcutaneous panniculitis-like T-cell lymphoma]]<br><ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016</ref><ref name="seer.cancer.gov2">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016</ref><ref name="pmid191959752">{{cite journal| author=Parveen Z, Thompson K| title=Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. | journal=Arch Pathol Lab Med | year= 2009 | volume= 133 | issue= 2 | pages= 303-8 | pmid=19195975 | doi=10.1043/1543-2165-133.2.303 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19195975  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Chills]]
+*[[Weight loss]]
+*[[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |Painless swellings on:
+* Extremities
+* Trunks
+| align="left" style="background:#F5F5F5;" + |
+* [[Myalgias]]
+| align="left" style="background:#F5F5F5;" + |Positive for:
+* BetaF1
+* [[CD8]]
+* [[CD30]]
+* [[CLA]]
+* T-cell intracellular antigen (T1A1)
+* [[Perforin]]
+| align="left" style="background:#F5F5F5;" + |
+* Atypical lymphoid cells
+* [[Fat necrosis]]
+* [[Karyorrhexis]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Subcutaneous panniculitis-like T-cell lymphoma]] can mimic the following conditions making it difficult to diagnose
+* [[Panniculitis|Begnign panniculitis]]
+* [[Eczema]]
+* [[Cellulitis]]
+* [[Dermatitis]]
+|-
+! align="center" style="background:#DCDCDC;" + |[[Hepatosplenic T cell lymphoma|Hepatosplenic  T-cell lymphoma]]<br><ref name="pmid28058028">{{cite journal| author=van de Meeberg MM, Derikx LA, Sinnige HA, Nooijen P, Schipper DL, Nissen LH| title=Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy. | journal=World J Gastroenterol | year= 2016 | volume= 22 | issue= 47 | pages= 10465-10470 | pmid=28058028 | doi=10.3748/wjg.v22.i47.10465 | pmc=5175260 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28058028  }}</ref><ref name="ShiWang2015">{{cite journal|last1=Shi|first1=Yang|last2=Wang|first2=Endi|title=Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer–Cell Neoplasms|journal=Archives of Pathology & Laboratory Medicine|volume=139|issue=9|year=2015|pages=1173–1180|issn=0003-9985|doi=10.5858/arpa.2014-0079-RS}}</ref><ref name="pmid22379294">{{cite journal| author=Alsohaibani FI, Abdulla MA, Fagih MM| title=Hepatosplenic T-cell lymphoma. | journal=Indian J Hematol Blood Transfus | year= 2011 | volume= 27 | issue= 1 | pages= 39-42 | pmid=22379294 | doi=10.1007/s12288-010-0051-1 | pmc=3102508 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22379294  }}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell Lymphoma
+* [[Inflammatory bowel disease]]
+* Organ transplant patients (reciever)
+* [[Immunosuppresive drug|Immunosuppresent medications]]
+* [[Thiopurine|Thiopurines]]
+* [[Infliximab]]
+* [[Cyclophosphamide]]
+* [[Vincristine sulfate|Vincristine]]
+* [[Doxorubicin hydrochloride|Doxorubicin]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Fatigue]]
+* [[Weight loss]]
+* [[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |Painless swelling in :
+* [[Neck masses|Neck]]
+* [[Axilla]]
+* [[Groin]]
+* [[Chest|Thorax]]
+* [[Abdominal mass|Abdomen]]
+| align="center" style="background:#F5F5F5;" + |
+* [[Skin rash]]
+* [[Chest pain]]
+* [[Abdominal pain]]
+* [[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD2]]
+* [[CD3 (immunology)|CD3]]
+* [[CD7]]
+* [[CD16]]
+* CD56  or [[CD57]]
+* [[TIA1]]
+* [[Granzyme|Granzyme m]]
+* [[Fas ligand]]
+| align="left" style="background:#F5F5F5;" + |
+* Intermediate sized  tumor cells with clear cytoplasm,  oval nuclei, slightly dispersed condensed chromatin, inconspicuous nucleoli
+| align="left" style="background:#F5F5F5;" + |
+* Patients with inflammatory bowel diseases taking immunosuppressant and anti-tumor necrosis factor-α agent are also have a higher risk for developing [[hepatosplenic  T-cell lymphoma]]
+|-
+! align="center" style="background:#DCDCDC;" + |[[Enteropathy-associated T-cell lymphoma|Enteropathy-type intestinal T-cell lymphoma]]<br><ref name="DelabieHolte2011">{{cite journal|last1=Delabie|first1=J.|last2=Holte|first2=H.|last3=Vose|first3=J. M.|last4=Ullrich|first4=F.|last5=Jaffe|first5=E. S.|last6=Savage|first6=K. J.|last7=Connors|first7=J. M.|last8=Rimsza|first8=L.|last9=Harris|first9=N. L.|last10=Muller-Hermelink|first10=K.|last11=Rudiger|first11=T.|last12=Coiffier|first12=B.|last13=Gascoyne|first13=R. D.|last14=Berger|first14=F.|last15=Tobinai|first15=K.|last16=Au|first16=W. Y.|last17=Liang|first17=R.|last18=Montserrat|first18=E.|last19=Hochberg|first19=E. P.|last20=Pileri|first20=S.|last21=Federico|first21=M.|last22=Nathwani|first22=B.|last23=Armitage|first23=J. O.|last24=Weisenburger|first24=D. D.|title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project|journal=Blood|volume=118|issue=1|year=2011|pages=148–155|issn=0006-4971|doi=10.1182/blood-2011-02-335216}}</ref><ref name="pmid22943012">{{cite journal |vauthors=Bautista-Quach MA, Ake CD, Chen M, Wang J |title=Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features |journal=J Gastrointest Oncol |volume=3 |issue=3 |pages=209–25 |date=September 2012 |pmid=22943012 |pmc=3418529 |doi=10.3978/j.issn.2078-6891.2012.024 |url=}}</ref><ref name="cancer.gov">Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+*[[Fever]]
+*[[Weight loss]]
+*[[Night sweats]]
+*[[Diarrhea]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="left" style="background:#F5F5F5;" + |Painless swelling in the :
+* [[Neck masses|Neck]]
+* [[axilla]]
+* [[groin]]
+* [[thorax]],
+* [[abdomen]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Skin rash]]
+* [[Chest pain]]
+* [[Abdominal pain]]
+* [[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |
+* [[CD3]]+
+* [[CD4]]-
+* [[CD5]]-
+* [[CD7]]+
+* [[CD30]]+
+* [[CD56]]+
+* [[CD103]]+
+* [[CD8]]+/-
+* TCR beta+/-
+| align="left" style="background:#F5F5F5;" + |
+*Lymphomatous infiltrate with [[neoplastic]] large cells
+*May have pleomorphic, multinucleated cells
+*Adjacent mucosa shows villous atrophy, crypt [[hyperplasia]], increased inflammatory cells
+*[[Lymphocytosis]]
+| align="left" style="background:#F5F5F5;" + |
+* Mostly diagnosed in the small intestine
+|-
+! align="center" style="background:#DCDCDC;" + |Extranodal T-cell lymphoma, nasal type<br><ref name="Hindawi">Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016</ref><ref name="cancer.gov2">Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 02, 2016</ref><ref name="Hindawi2">Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Weight loss]]
+* [[Night sweats]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |Painless swelling in :
+* [[Neck masses|Neck]]
+* [[Axilla]]
+* [[Groin]]
+* [[Chest|Thorax]]
+* [[Abdominal mass|Abdomen]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Rash|Skin rash]]
+* [[Chest pain]]
+* [[Abdominal pain]]
+* [[Bone pain]]
+| align="left" style="background:#F5F5F5;" + |
+* Cytoplasmic [[CD3]] epsilon
+* [[Granzyme|Granzyme B]]
+* [[Perforin]]
+* [[CD2]]
+* [[CD56]]
+| align="left" style="background:#F5F5F5;" + |
+* Medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells
+* [[Lymphoma|Lymphoma cells]] may be admixed with a polymorphic infiltrate of non-neoplastic inflammatory cells
+| align="left" style="background:#F5F5F5;" + |
+* [[Proptosis/exophthalmos|Protrusion of eye]]
+* [[Swollen face|Swelling of the face]]
+* Discharge from the nose
+* [[Epistaxis|Nose bleeds]]
+* [[Nasal congestion|Blockage of the nasal passages]]
+|-
+! align="center" style="background:#DCDCDC;" + |Angioimmunoblastic T-cell lymphoma<br><ref name="who1">{{cite book |last=Swerdlow |first=S.H. |last2=Campo |first2=E. |last3=Harris |first3=N.L. |last4=Jaffe |first4=E.S. |last5=Pileri |first5=S.A. |last6=Stein |first6=H. |last7=Thiele |first7=J. |last8=Vardiman |first8=J.W |chapter=11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma |title=WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues |edition=4th |series=IARC WHO Classification of Tumours |volume=2 |publisher=IARC |year=2008 |isbn=9283224310 |url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002&content=1}}</ref><ref name="quin1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." '''Am J Surg Pathol'''. 1999 Oct;23(10):1233-40. PMID: 10524524</ref><ref name="seer">Angioimmunoblastic T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52dc/ Accessed on November 27, 2015</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+* [[Fever]]
+* [[Weight loss]]
+* [[Night sweats]]
+* [[Edema]]
+* [[Fatigue]]
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* Painless swelling in :
+* [[Neck masses|Neck]]
+* [[Axilla]]
+* [[Groin]]
+* [[Chest|Thorax]]
+* [[Abdominal mass|Abdomen]]
+| align="left" style="background:#F5F5F5;" + |
+* Skin rash
+* [[abdominal pain]]
+* [[bone pain]]
+* [[dark urine]]
+* [[shortness of breath]]
+* [[chronic pain]] and swelling of joints
+* Chest pain
+| align="left" style="background:#F5F5F5;" + |
+* [[CD2]]
+* [[CD3]]
+* [[CD4]]
+* [[CD5]]
+* [[CD10]]
+* [[CD20]]
+* Focal positivity for [[CXCL13]]
+| align="left" style="background:#F5F5F5;" + |
+* Leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes
+* Hyperplastic [[germinal centers]] and [[Reed-Sternberg cells]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Monoclonal gammopathy]]
+* [[Autoimmune hemolytic anemia]]
+|-
+! align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma, unspecified<br><ref name="pmid22760778">{{cite journal |vauthors=Lemonnier F, Couronné L, Parrens M, Jaïs JP, Travert M, Lamant L, Tournillac O, Rousset T, Fabiani B, Cairns RA, Mak T, Bastard C, Bernard OA, de Leval L, Gaulard P |title=Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters |journal=Blood |volume=120 |issue=7 |pages=1466–9 |date=August 2012 |pmid=22760778 |doi=10.1182/blood-2012-02-408542 |url=}}</ref><ref name="JhaGupta2017">{{cite journal|last1=Jha|first1=KunalKishor|last2=Gupta|first2=SureshK|last3=Saluja|first3=Harpreet|last4=Subedi|first4=Nuwadatta|title=Peripheral T-cell lymphoma, not otherwise specified|journal=Journal of Family Medicine and Primary Care|volume=6|issue=2|year=2017|pages=427|issn=2249-4863|doi=10.4103/jfmpc.jfmpc_323_16}}</ref>
+| align="left" style="background:#F5F5F5;" + |
+* Mature T-cell lymphoma
+| align="left" style="background:#F5F5F5;" + |
+* [[Fatigue]]
+* [[Fever]]
+* Frequent [[Infection|infections]]
+| align="center" style="background:#F5F5F5;" + | -
+| align="center" style="background:#F5F5F5;" + | +
+| align="center" style="background:#F5F5F5;" + | -
+| align="left" style="background:#F5F5F5;" + |
+* [[Lymphadenopathy]]
+* [[Hepatosplenomegaly]]
+| align="left" style="background:#F5F5F5;" + |
+* [[Lymphadenopathy]] (swollen )
+* [[Thirst]]
+* [[Nausea and vomiting|Nausea]]
+* [[Nausea and vomiting|Vomiting]]
+* Skin and bone abnormalities
+* Enlarged liver and/or spleen
+| align="left" style="background:#F5F5F5;" + |
+*[[CD2]]
+*[[CD3]]
+*[[TCR]]
+*β F1
+*Variable [[CD4]]
+*[[CD5]]
+*[[CD7]]
+*Occasional [[CD56]]
+*Cytotoxic granule expression
+| align="left" style="background:#F5F5F5;" + |
+*Clear cytoplasm, resemble [[Reed-Sternberg cell|Reed-Sternberg cells]], with irregular, pleomorphic, hyperchromatic or  nuclei
+*A lot of mitotic figures; very broad cytologic spectrum
+| align="left" style="background:#F5F5F5;" + |
+* Endogenous [[immunosupression]]
+* Patients may present with [[Pneumocystis jirovecii pneumonia|p.jiroveci]]  or [[Chickenpox|varicella zoster]] infection
+* Can be confused with other [[infectious]] or rheumatologic diseases
+|}
-==[[Hepatosplenic T cell lymphoma causes|Causes]]==
+==Epidemiology and Demographics==
+* The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year.
+* It occurs in younger group of patients, most cases falling in 20-40 years of age group.
+* Men are more affected than the females.
-==[[Hepatosplenic T cell lymphoma epidemiology and demographics|Epidemiology and Demographics]]==
+==Risk Factors==
+Common risk factors include:
+* [[Autoimmune diseases]]
+* Patients on immunosuppresant medications.
+* Patients on [[Chemotherapy|chemotherapy.]]
+* Inflammatory bowel disease
+* Organ transplant patients.
-==[[Hepatosplenic T cell lymphoma epidemiology and demographics|Risk Factors]]==
+==Screening==
+There is insufficient evidence to recommend routine screening for hepatosplenic T cell lymphoma.
-==[[Hepatosplenic T cell lymphoma screening|Screening]]==
+==Natural History, Complications, and Prognosis==
-==[[Hepatosplenic T cell lymphoma differential diagnosis|Differentiating Hepatosplenic T cell lymphoma from other Diseases]]==
+=== Natural history ===
+* Patients have a history of [[immunosupression]] such as inflammatory bowel disease under treatment or organ transplant.<ref name="pmid19237479">{{cite journal| author=Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE et al.| title=Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. | journal=Ann Oncol | year= 2009 | volume= 20 | issue= 6 | pages= 1080-5 | pmid=19237479 | doi=10.1093/annonc/mdn751 | pmc=4092251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19237479  }} </ref>
+* The mean age group is 35 years and most of the patients are males.
+* Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no [[Lymphadenopathy|lymphadenopathy.]]
+* Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
+* If left untreated, patients can develop [[Hepatic failure|liver failure]], [[pancytopenia]], or [[spleen rupture]].
-==[[Hepatosplenic T cell lymphoma natural history|Natural History, Complications and Prognosis]]==
+=== Complications ===
+* [[Hepatomegaly]]
+* [[Hepatic failure]]
+* [[Portal vein thrombosis]]
+* [[Splenomegaly]]
+* [[Splenic infarction]]
+* Spleen rupture
+* [[Splenic vein thrombosis]]
+* [[Anemia]]
+* [[Thrombocytopenia]]
+* [[Neutropenia]]
+* Neurological dysfunction if metastasizes to brain.
+* [[Gastrointestinal perforation|Intestinal perforation]]
+* [[Intestinal obstruction]]
+*
+=== Prognosis ===
+* The prognosis is very poor, with patients dying within 2-3 years of diagnosis even after recieving treatment<ref name="pmid26872013">{{cite journal| author=Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y et al.| title=Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases. | journal=Am J Surg Pathol | year= 2016 | volume= 40 | issue= 5 | pages= 676-88 | pmid=26872013 | doi=10.1097/PAS.0000000000000614 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26872013  }} </ref>.
 ==Diagnosis==
-[[Hepatosplenic T cell lymphoma staging|Staging]] | [[Hepatosplenic T cell lymphoma history and symptoms|History and Symptoms]] | [[Hepatosplenic T cell lymphoma physical examination|Physical Examination]] | [[Hepatosplenic T cell lymphoma laboratory tests|Laboratory Findings]] | [[Hepatosplenic T cell lymphoma biopsy|Biopsy]] | [[Hepatosplenic T cell lymphoma CT|CT]] | [[Hepatosplenic T cell lymphoma MRI|MRI]] | [[Hepatosplenic T cell lymphoma ultrasound|Ultrasound]] | [[Hepatosplenic T cell lymphoma other imaging findings|Other Imaging Findings]] | [[Hepatosplenic T cell lymphoma other diagnostic studies|Other Diagnostic Studies]]
+===Diagnostic Study of Choice===
+* Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic T cell lymphoma<ref name="pmid8141877">{{cite journal| author=International Non-Hodgkin's Lymphoma Prognostic Factors Project| title=A predictive model for aggressive non-Hodgkin's lymphoma. | journal=N Engl J Med | year= 1993 | volume= 329 | issue= 14 | pages= 987-94 | pmid=8141877 | doi=10.1056/NEJM199309303291402 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8141877  }} </ref>.
+* CT scan and PET scan are used to assess the spread of the lymphoma.
+=== Symptoms ===
+The most common symtoms are<ref name="pmid12419753">{{cite journal| author=Saito T, Matsuno Y, Tanosaki R, Watanabe T, Kobayashi Y, Tobinai K| title=Gamma delta T-cell neoplasms: a clinicopathological study of 11 cases. | journal=Ann Oncol | year= 2002 | volume= 13 | issue= 11 | pages= 1792-8 | pmid=12419753 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12419753  }} </ref>:
+* Fever
+* Weight loss
+* Night sweats
+* Pain abdomen
+* [[Jaundice]]
+* Fatigue
+* Recurrent infections
+* Bleeding
+===Physical Examination===
+=== Temperature ===
+* [[Fever]] is often present
+=== Skin ===
+* [[Rash]]
+=== Thorax ===
+* [[Pleural effusion]]
+* Chest tenderness
+=== Abdomen ===
+* [[Splenomegaly]]
+* [[Hepatomegaly]]
+* [[Ascites]]
+* Abdomen tenderness<ref name="pmid19237479">{{cite journal| author=Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE et al.| title=Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. | journal=Ann Oncol | year= 2009 | volume= 20 | issue= 6 | pages= 1080-5 | pmid=19237479 | doi=10.1093/annonc/mdn751 | pmc=4092251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19237479  }} </ref>
+=== Extremities ===
+* Bone tenderness<ref name="pmid19237479">{{cite journal| author=Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE et al.| title=Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. | journal=Ann Oncol | year= 2009 | volume= 20 | issue= 6 | pages= 1080-5 | pmid=19237479 | doi=10.1093/annonc/mdn751 | pmc=4092251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19237479  }} </ref>
+===Laboratory Findings===
+* Biopsy of the tumor:
+** [[Histology]] - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
+** [[Flow cytometry]] and [[immunophenotyping]] - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression<ref name="pmid9496260">{{cite journal| author=Boulland ML, Kanavaros P, Wechsler J, Casiraghi O, Gaulard P| title=Cytotoxic protein expression in natural killer cell lymphomas and in alpha beta and gamma delta peripheral T-cell lymphomas. | journal=J Pathol | year= 1997 | volume= 183 | issue= 4 | pages= 432-9 | pmid=9496260 | doi=10.1002/(SICI)1096-9896(199712)183:4<432::AID-PATH942>3.0.CO;2-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9496260  }} </ref>.
+** [[Karyotype|Karyotyping]] - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome<ref name="pmid8751461">{{cite journal| author=Jonveaux P, Daniel MT, Martel V, Maarek O, Berger R| title=Isochromosome 7q and trisomy 8 are consistent primary, non-random chromosomal abnormalities associated with hepatosplenic T gamma/delta lymphoma. | journal=Leukemia | year= 1996 | volume= 10 | issue= 9 | pages= 1453-5 | pmid=8751461 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8751461  }} </ref>.
+* Bone marrow biopsy; The bone marrow is characterized by [[Cancer|neoplastic]] cells in the sinusoids.
+* Complete blood count; [[Pancytopenia]]
+* Liver function tests: Deranged [[Liver function tests|LFT]]
+** Elevated [[Transaminase|transaminases]]
+** [[Jaundice|Hyperbilirubinemia]]
+** [[Hypoproteinemia]]
+** Elevated [[alkaline phosphatase]]
+** Elevated [[PT]]/[[Prothrombin time|INR]]
+===Electrocardiogram===
+There are no ECG findings associated with hepatosplenic T cell lymphoma.
+===X-ray===
+There are no x-ray findings associated with hepatosplenic T cell lymphoma but [[pleural effusion]] might be present.
+===Echocardiography or Ultrasound===
+* There are no echocardiography findings associated with hepatosplenic T cell lymphoma.
+* On ultrasound of abdomen:
+** [[Hepatomegaly]]
+** [[Splenomegaly]]
+===CT scan===
+Tumor mass can be seen in [[liver]] or [[spleen]] or both.
+===MRI===
+Tumor mass can be seen in [[liver]] or [[spleen]] or both.
+===Other Imaging Findings===
+PET scan: On PET scan, tumor mass can be seen in liver or spleen or if it has metastasized to any other organ.
 ==Treatment==
-[[Hepatosplenic T cell lymphoma medical therapy|Medical Therapy]] | [[Hepatosplenic T cell lymphoma surgery|Surgery]] | [[Hepatosplenic T cell lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hepatosplenic T cell lymphoma future or investigational therapies|Future or Investigational Therapies]]
+===Medical Therapy===
+* [[Induction (biology)|Induction]] therapy<ref name="pmid20738307">{{cite journal| author=Simon A, Peoch M, Casassus P, Deconinck E, Colombat P, Desablens B et al.| title=Upfront VIP-reinforced-ABVD (VIP-rABVD) is not superior to CHOP/21 in newly diagnosed peripheral T cell lymphoma. Results of the randomized phase III trial GOELAMS-LTP95. | journal=Br J Haematol | year= 2010 | volume= 151 | issue= 2 | pages= 159-66 | pmid=20738307 | doi=10.1111/j.1365-2141.2010.08329.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20738307  }} </ref>
+** [[Cyclophosphamide]]
+** [[Vincristine sulfate|Vincristine]]
+** [[Doxorubicin hydrochloride|Doxorubicin]]
+** [[Prednisolone]]
+* [[Consolidation (medicine)|Consolidation]] therapy
+** [[Radiation therapy]]
+** Allogenic [[Hematopoietic stem cell transplantation|bone marrow transplant]]<ref name="pmid28222648">{{cite journal| author=Han X, Zhang W, Zhou D, Ruan J, Duan M, Zhu T et al.| title=Autologous stem cell transplantation as frontline strategy for peripheral T-cell lymphoma: A single-centre experience. | journal=J Int Med Res | year= 2017 | volume= 45 | issue= 1 | pages= 290-302 | pmid=28222648 | doi=10.1177/0300060516676725 | pmc=5536587 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28222648  }} </ref>
+** Autologous [[Hematopoietic stem cell transplantation|bone marrow transplant]]
+* Additional medications given during chemotherapy:
+** [[Acyclovir]]
+** [[Fluconazole]]
+** [[Sulfamethoxazole-Trimethoprim|TMP/sulphamethaxozole]]
+===Surgery===
+Surgical intervention is not recommended for the management of hepaosplenic T cell lymphoma.
+===Primary Prevention===
+There are no established measures for the primary prevention of hepatosplenic T cell lymphoma.
-{{Hematological malignancy histology}}
+===Secondary Prevention===
+There are no established measures for the secondary prevention of hepatosplenic T cell lymphoma.
-[[Category:Hematology|Hepatosplenic T-cell lymphoma]]
+==References==
-[[Category:Types of cancer|Hepatosplenic T-cell lymphoma]]
+{{reflist|2}}
-[[Category:Oncology]]
-[[Category:Disease]]
-[[Category:Gastroenterology]]
-[[Category:Hepatology]]
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