|
|
Line 16: |
Line 16: |
| *It supports rapid B-cell proliferation for [[immunoglobulin]] affinity maturation and production of antibody diversity through two processes know as [[somatic hypermutation]] (SHM) and [[Class switching|immunoglobulin class switching]]. | | *It supports rapid B-cell proliferation for [[immunoglobulin]] affinity maturation and production of antibody diversity through two processes know as [[somatic hypermutation]] (SHM) and [[Class switching|immunoglobulin class switching]]. |
| *Both of these processes require rapid cell turnover and multiple double stranded [[DNA]] breaks, which is error-prone. | | *Both of these processes require rapid cell turnover and multiple double stranded [[DNA]] breaks, which is error-prone. |
| *Somatically acquired genetic alterations ( mainly [[Chromosomal translocation|translocations]]) of these processes is probably the underlying cause of lymphomagenesis.<ref name="pmid25712152">{{cite journal |vauthors=Basso K, Dalla-Favera R |title=Germinal centres and B cell lymphomagenesis |journal=Nat. Rev. Immunol. |volume=15 |issue=3 |pages=172–84 |date=March 2015 |pmid=25712152 |doi=10.1038/nri3814 |url=}}</ref><ref name="pmid10648419">{{cite journal |vauthors=Nardini E, Aiello A, Giardini R, Colnaghi MI, Ménard S, Balsari A |title=Detection of aberrant isotype switch recombination in low-grade and high-grade gastric MALT lymphomas |journal=Blood |volume=95 |issue=3 |pages=1032–8 |date=February 2000 |pmid=10648419 |doi= |url=}}</ref><ref name="pmid18097447">{{cite journal |vauthors=Klein U, Dalla-Favera R |title=Germinal centres: role in B-cell physiology and malignancy |journal=Nat. Rev. Immunol. |volume=8 |issue=1 |pages=22–33 |date=January 2008 |pmid=18097447 |doi=10.1038/nri2217 |url=}}</ref> | | *Somatically acquired genetic alterations ( mainly [[Chromosomal translocation|translocations]]) of these processes is probably the underlying cause of lymphomagenesis. |
|
| |
|
| * The major subtypes of non-hodgkin lymphoma include the following:<ref name="pmid21261684">{{cite journal| author=Coupland SE| title=The challenge of the microenvironment in B-cell lymphomas. | journal=Histopathology | year= 2011 | volume= 58 | issue= 1 | pages= 69-80 | pmid=21261684 | doi=10.1111/j.1365-2559.2010.03706.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21261684 }}</ref><ref>{{cite journal|doi=10.1182/blood-2016- 01-643569}}</ref> | | * The major subtypes of non-hodgkin lymphoma include the following: |
| ** Mature B-cell neoplasms: | | ** Mature B-cell neoplasms: |
| *** [[Burkitt's lymphoma|Burkitt lymphoma]] | | *** [[Burkitt's lymphoma|Burkitt lymphoma]] |
Line 36: |
Line 36: |
| ==Genetics== | | ==Genetics== |
|
| |
|
| Different subtypes of non Hodgkin lymphoma and their genetic involvements::<ref>Burkitt's Lymphoma. Wikibooks. https://en.wikibooks.org/wiki/Radiation_Oncology/NHL/Burkitt_lymphoma#Pathology Accessed on October,5 2015</ref><ref>{{cite web|title=NIH study shows Burkitt lymphoma is molecularly distinct from other lymphomas|url=http://cancer.gov/newscenter/newsfromnci/2012/BurkittReleaseStaudt|publisher=National Cancer Institute}}</ref><ref>Staudt L, et al. Burkitt Lymphoma Pathogenesis and Therapeutic Targets from Structural and Functional Genomics. Nature. August 12, 2012 {{DOI|10.1038/nature11378}}</ref><ref name="pmid21804550">{{cite journal |vauthors=Pasqualucci L, Trifonov V, Fabbri G, Ma J, Rossi D, Chiarenza A, Wells VA, Grunn A, Messina M, Elliot O, Chan J, Bhagat G, Chadburn A, Gaidano G, Mullighan CG, Rabadan R, Dalla-Favera R |title=Analysis of the coding genome of diffuse large B-cell lymphoma |journal=Nat. Genet. |volume=43 |issue=9 |pages=830–7 |date=July 2011 |pmid=21804550 |pmc=3297422 |doi=10.1038/ng.892 |url=}}</ref><ref name="pmid26573234">{{cite journal |vauthors=Ye Q, Xu-Monette ZY, Tzankov A, Deng L, Wang X, Manyam GC, Visco C, Montes-Moreno S, Zhang L, Dybkær K, Chiu A, Orazi A, Zu Y, Bhagat G, Richards KL, Hsi ED, Choi WW, van Krieken JH, Huh J, Ponzoni M, Ferreri AJ, Parsons BM, Møller MB, Piris MA, Winter JN, Medeiros LJ, Hu S, Young KH |title=Prognostic impact of concurrent MYC and BCL6 rearrangements and expression in de novo diffuse large B-cell lymphoma |journal=Oncotarget |volume=7 |issue=3 |pages=2401–16 |date=January 2016 |pmid=26573234 |pmc=4823044 |doi=10.18632/oncotarget.6262 |url=}}</ref><ref name="pmid28379189">{{cite journal |vauthors=Nguyen L, Papenhausen P, Shao H |title=The Role of c-MYC in B-Cell Lymphomas: Diagnostic and Molecular Aspects |journal=Genes (Basel) |volume=8 |issue=4 |pages= |date=April 2017 |pmid=28379189 |pmc=5406863 |doi=10.3390/genes8040116 |url=}}</ref><ref name="OffitCoco1994">{{cite journal|last1=Offit|first1=Kenneth|last2=Coco|first2=Francesco Lo|last3=Louie|first3=Diane C.|last4=Parsa|first4=Nasser Z.|last5=Leung|first5=Denis|last6=Portlock|first6=Carol|last7=Ye|first7=Bihui H.|last8=Lista|first8=Florigio|last9=Filippa|first9=Daniel A.|last10=Rosenbaum|first10=Ayala|last11=Ladanyi|first11=Marc|last12=Jhanwar|first12=Suresh|last13=Dalla-Favera|first13=Riccardo|last14=Chaganti|first14=R.S.K.|title=Rearrangement of the bcl-6 Gene as a Prognostic Marker in Diffuse Large-Cell Lymphoma|journal=New England Journal of Medicine|volume=331|issue=2|year=1994|pages=74–80|issn=0028-4793|doi=10.1056/NEJM199407143310202}}</ref><ref name="pmid9787151">{{cite journal |vauthors=Kramer MH, Hermans J, Wijburg E, Philippo K, Geelen E, van Krieken JH, de Jong D, Maartense E, Schuuring E, Kluin PM |title=Clinical relevance of BCL2, BCL6, and MYC rearrangements in diffuse large B-cell lymphoma |journal=Blood |volume=92 |issue=9 |pages=3152–62 |date=November 1998 |pmid=9787151 |doi= |url=}}</ref><ref>{{Cite journal | | Different subtypes of non Hodgkin lymphoma and their genetic involvements:: |
| | 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>
| |
|
| |
|
| {| class="wikitable" | | {| class="wikitable" |
Line 359: |
Line 348: |
| |} | | |} |
|
| |
|
| == Differential diagnosis of Lymphocytosis ==
| |
|
| |
| {| class="wikitable"
| |
| |+
| |
| !
| |
| !Pathophyisiology
| |
| !Symptoms
| |
| !History
| |
| !Physical Examination
| |
| !Laboratory Findings
| |
| |-
| |
| |AML
| |
| |
| |
| * Mutation of myeloblast freezes the cell in its immature state and prevent [[cellular differentiation|differentiation]].
| |
| |
| |
| * Persistent or frequent [[infections]].
| |
| * [[Anemia]] leads to fatigue, paleness, and shortness of breath.
| |
| * Thrombocytopenia leads to bruising or bleeding with minor trauma.
| |
| |
| |
| * History of pre-existing hematological disorder (e.g [[aplastic anemia]], [[PNH]], [[myelofibrosis]])
| |
| * History of exposure to anti-cancer [[chemotherapy]] agents especially alkylating agents
| |
| * History of exposure to [[ionizing radiation]]
| |
| * History of occupational exposure to [[benzene]] and other [[aromatic hydrocarbons]]
| |
| * History of any [[congenital]] disorders (e.g [[Down syndrome]], [[Bloom syndrome]])
| |
| |
| |
| * Bone tenderness
| |
| * Skin manifestations
| |
| |
| |
| * Immature Myeloblasts on blood smear
| |
| * Flow cytometry
| |
| * +Aur Rods
| |
| |-
| |
| |ALL
| |
| |
| |
| * Arrest of [[lymphoblasts]].
| |
| * [[Chromosomal translocations]] involved
| |
| ** 9 and 22, t(9;22) (q34;q11.2) ''BCR-ABL1''
| |
| ** 12 and 21, t(12;21)(p13;q22) ''TEL-AML1''
| |
| ** 5 and 14, t(5;14)(q31;q32)''IL3-IGH''
| |
| ** 1 and 19 t(1;19)(q23;p13.3) ''TCF3-PBX1''
| |
| |
| |
| * Generalised weakness and [[Fatigue (physical)|fatigue]]
| |
| * Frequent or unexplained [[fever]] and [[Infection|infections]]
| |
| * [[Weight loss]] and/or loss of appetite
| |
| * Excessive [[bruising]], [[Hemorrhage|bleeding]] from wounds, [[Nosebleed|nosebleeds]], [[petechiae]], [[bone pain]], [[Joint pain|joint pains]] and [[dyspnea]].
| |
| |
| |
| * History of cancer
| |
| * History of drug exposure
| |
| |
| |
| * [[Lymphadenopathy]]
| |
| * [[Hepatomegaly|Hepato-splenomegaly]]
| |
|
| |
| * [[Stridor]]
| |
| * [[Pallor]]
| |
| * P[[Petechiae|etechiae]]
| |
| * B[[Bruising|ruising]]
| |
| * [[Papilledema]]
| |
| * Nuchar rigidity
| |
| * [[Cranial nerve palsy]]
| |
| * Testicular enlargement
| |
| |
| |
| * [[Eosinophilia]]
| |
| * [[Lymphocytosis]]
| |
| * Decreased erythrocytes production
| |
| * [[Thrombocytopenia]].
| |
| * Chemistry panels with altered levels of [[uric acid]], [[creatinine]], [[blood urea nitrogen]], [[potassium]], [[phosphate]], [[calcium]], [[bilirubin]], [[hepatic transaminases]] and [[ferritin]].
| |
| * A [[Lumbar puncture|spinal tap]] will tell if the spinal column and [[Central nervous system|brain]] has been invaded.
| |
| |-
| |
| |CML
| |
| |
| |
| * Myeloproliferative expansion of pluripotent stem cells.
| |
| * Philadelphia chromosome resulting from the reciprocal t(9;22)(q34;q11.2)
| |
| ** Resulting in a derivative 9q+ and a small 22q-. results in a ''BCR-ABL'' fusion gene
| |
| ** Ativates numerous downstream targets including
| |
| *** ''c-myc''
| |
| *** ''Akt''
| |
| *** ''Jun'',
| |
| |
| |
| * Insidious in onset
| |
| * Nonspecific symptoms of fatigue and weight loss.
| |
| * Early satiety and decreased food intake due to splenic compression of stomach
| |
| * Low-grade fever and excessive sweating
| |
| |
| |
| |
| |
| * Splenomegaly
| |
| ** Correlates with granulocyte counts
| |
| * Findings of leukostasis and hyperviscosity.
| |
| * Funduscopy may show papilledema, venous obstruction, and hemorrhages.
| |
| |
| |
| * WBC counts, exceeding 300,000-600,000 cells/μL
| |
| * Elevated alkaline phosphatase (ALP)
| |
| * Philadelphia (Ph1) chromosome\
| |
| *
| |
| |-
| |
| |CLL
| |
| |
| |
| * Clonal B cells arrested in the B-cell differentiation pathway,
| |
| * [[Genetic mutation|Genetic mutations]] that promote both [[malignant]] leukemic proliferation and [[apoptotic]] resistance of mature B cells.
| |
| * Structural [[Genetic mutation|genetic mutations]] involved in the pathogenesis of chronic lymphocytic leukemia include [[chromosome]] 13q deletion, chromosome 17p deletion, and chromosome 11q deletion.
| |
| ** ''SF3B1'' gene located on [[chromosome 2]]
| |
| ** ''FBXW7'' gene located on [[chromosome 4]]
| |
| ** ''MYD88'' gene located on [[chromosome 3]]
| |
| ** ''TP53'' gene located on [[chromosome 7]]
| |
| ** ''NOTCH1'' gene located on [[chromosome 9]]
| |
| ** ''ATM'' gene located on [[chromosome 11]]
| |
| ** ''CHD2'' gene located on [[chromosome 15]]
| |
| |
| |
| * [[Fever]]
| |
| * Recurrent [[bleeding]]
| |
| * [[Weight loss]]
| |
| * [[Muscle wasting]]
| |
| * Generalized [[weakness]]
| |
| * Anorexia
| |
| * [[Night sweats]]
| |
| * [[Abdominal pain]]
| |
| * Recurrent [[Infection|infections]]
| |
| |
| |
| * Review family history for members with positive history of the disease
| |
| * Review occupational history related to farming
| |
| * Review any exposure to herbicides or insecticides
| |
| |
| |
| * Skin [[pallor]]
| |
| * Palpable [[cervical]] [[Lymph node|lymph nodes]]
| |
| * [[Hepatomegaly]].
| |
| |
| |
| * Monoclonality of kappa and lambda producing [[B cell|B cells]]
| |
| * Presence of smudge cells
| |
|
| |
| * CBC
| |
| ** Absolute [[lymphocytosis]] (>5000 cells/μl)
| |
| ** Decreased [[hemoglobin]] concentration
| |
| ** Decreased [[Platelet|platelets]] count
| |
|
| |
| * Express [[CD19]], [[CD20]], [[CD23]], and [[CD5]] on the [[cell]] surface
| |
| |}
| |
| {| class="wikitable"
| |
| |+
| |
| !
| |
| ! rowspan="2" |Pathophysiology
| |
| ! rowspan="2" |Symptoms
| |
| ! rowspan="2" |History
| |
| ! rowspan="2" |Physical Examination
| |
| ! colspan="3" |Laboratory Findings
| |
| |
| |
| |-
| |
| !
| |
| !CBC
| |
| !Blood smear
| |
| !Immunophenotype
| |
| !
| |
| |-
| |
| |'''Monoclonal B lymphocytosis'''
| |
| |
| |
| * Monoclonal population of B lymphocytes <5000 cells/microL
| |
| |
| |
| * Without other features of
| |
| ** Lymphadenopathy
| |
| ** Organomegaly
| |
| ** Extra-medullary involvement
| |
| |
| |
| * Active or prior infections
| |
| * History of hematologic malignancy
| |
| * Medications
| |
| * Family history of chronic lymphocytic leukemia (CLL)
| |
| |
| |
| * Fever
| |
| * Lymphadenopathy
| |
| * Hepatosplenomegaly
| |
| * Joint redness
| |
| * Abdominal pain
| |
| * Lung findings.
| |
| |
| |
| * Lymphocytosis ≥4000 lymphocytes/microL
| |
| *
| |
| |
| |
| * Lymphocytes in MBL have no distinguishing appearance
| |
| * Appear as small, mature mononuclear cells.
| |
| |
| |
| * CD19, CD20, and CD23
| |
| |
| |
| * Does not require bone marrow examination or imaging for diagnosis
| |
| |-
| |
| |'''Congenital B cell lymphocytosis'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| *
| |
| |
| |
| |-
| |
| |'''Large granular lymphocyte leukemia'''
| |
| |
| |
| * T-cell (T-LGL)
| |
| * Natural killer cell (NK-LGL)
| |
| |
| |
| * Recurrent infections
| |
| * Fever
| |
| * Night sweats
| |
| * Unintended weight loss
| |
| * Lymphadenopathy
| |
| |
| |
| |
| |
| |
| |
| * Pancytopenia
| |
| * Splenomegaly
| |
| |
| |
| |
| |
| * CD3, CD57, CD56
| |
| * CD3-, CD56+
| |
| |
| |
| |-
| |
| |'''Chronic lymphocytic leukemia'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Sezary syndrome'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Mantle cell lymphoma'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Follicular lymphoma'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Splenic marginal zone lymphoma'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Acute lymphoblastic leukemia'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Acute Promyelocytic Leukemia'''
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |-
| |
| |'''Diffuse Large Cell Lymphoma'''
| |
| |
| |
| |
| |
| * Enlarged painful lymph node
| |
| *
| |
| |
| |
| * Neurological or gastrointestinal manifestations
| |
| * History of environmental and/or infectious disease exposure
| |
| |
| |
| * Lymphadenopathy
| |
| * Splenomegaly
| |
| * Low-grade fever
| |
| * Pedal edema:
| |
| |
| |
| |
| |
| |
| |
| |
| |
| |}
| |
|
| |
| == Differential for Eosinophilia ==
| |
| {| class="wikitable"
| |
| |+
| |
| !
| |
| !Pathophysiology
| |
| !Symptoms
| |
| !History
| |
| !Physical Examination
| |
| !Laboratory Findings
| |
| |-
| |
| |Parasitic Infections
| |
| |
| |
| * Egg deposition
| |
| * Liberation of [[antigens]] of adult worms and eggs
| |
| * Strong [[inflammatory response]] characterized by high levels of pro-inflammatory [[cytokines]]
| |
| ** I[[IL-1|nterleukins 1]], [[Interleukin 6|6]], [[TNF-α|tumor necrosis factor-α]], and circulating [[immune complexes]] participates in the pathogenesis of the acute phase of the [[disease]]
| |
| |Vary depending on the organism
| |
| * GI
| |
| * STD
| |
| * Neurological
| |
| * Swollen lymph nodes and muscle aches or pains
| |
| |
| |
| * History of travel
| |
| |
| |
| * Rash
| |
| * Fever
| |
| * Lymphadenopathy
| |
| * Ulcers
| |
| |
| |
| * +Stool examination
| |
| * + Serologic testing
| |
| * Urinalysis
| |
| |-
| |
| |Allergy/ Atopic Diseases
| |
| |
| |
| * Allergic hypersensitivity
| |
| ** IgE stimulation
| |
| |'''Systemic anaphylaxis'''
| |
| * Reaction occurs within minutes
| |
| * Leading to symptomatology such as
| |
| ** Acute asthma
| |
| ** Laryngeal edema
| |
| ** Diarrhea
| |
| ** Urticaria
| |
| ** Shock.
| |
| * Classic examples are penicillin allergy and bee sting allergy.
| |
| '''Local anaphylaxis (atopy)'''
| |
| * About 10% of people have "atopy" and are easily sensitized to allergens that cause a localized reaction when inhaled or ingested.
| |
| ** Hay fever
| |
| ** Hives, asthma.
| |
| * Classic examples are food allergies and hay fever to ragweed pollen
| |
|
| |
| *
| |
| |
| |
| * History consistent with allergy and specific allergens
| |
| |
| |
| * [[Eczema]] ([[atopic dermatitis]])
| |
| * [[Allergic conjunctivitis]]
| |
| * [[Allergic rhinitis]]
| |
| * [[Asthma]]
| |
|
| |
| *
| |
| |
| |
| * Increase in eosinophils
| |
| * Serum tryptase positive reaction
| |
| * Testing for specific IgE antigen.
| |
| |-
| |
| |Hypereosinophilic syndromes (HES)
| |
| |
| |
| * Activation of tyrosine kinases
| |
| ** Clonal eosinophilic proliferation
| |
| ** Overproduction of eosinophilopoietic cytokines.
| |
| |
| |
| * [[Shortness of breath]]
| |
| * [[Skin rash]]
| |
| * [[Cough]]
| |
| * [[Diarrhea]]
| |
| * [[Myalgias]]
| |
| * [[Fatigue]]
| |
| * [[Weight-loss]]
| |
| |
| |
| |
| |
| * [[Skin rash]]
| |
| :* Thickening of the skin ([[lichenification]])
| |
| :* Eczema (flexural areas)
| |
| :* [[Dermographism]]
| |
| * [[Low-grade fever]]
| |
| * [[Raynaud phenomenon]]
| |
| * [[Wheezing]]
| |
| |
| |
| * Persistently [[Eosinophilia|elevated eosinophil count]] (≥ 1500 eosinophils/mm³) in the blood
| |
| :* At least six months without any recognizable cause
| |
| :* Involvement of either the [[heart]], [[nervous system]], or [[bone marrow]].
| |
| |-
| |
| |Acute myelogenous leukemias
| |
| |
| |
| * Mutation of myeloblast freezes the cell in its immature state and prevent [[cellular differentiation|differentiation]].
| |
| |
| |
| * Persistent or frequent [[infections]].
| |
| * [[Anemia]] leads to fatigue, paleness, and shortness of breath.
| |
| * Thrombocytopenia leads to bruising or bleeding with minor trauma.
| |
| |
| |
| * History of pre-existing hematological disorder (e.g [[aplastic anemia]], [[PNH]], [[myelofibrosis]])
| |
| * History of exposure to anti-cancer [[chemotherapy]] agents especially alkylating agents
| |
| * History of exposure to [[ionizing radiation]]
| |
| * History of occupational exposure to [[benzene]] and other [[aromatic hydrocarbons]]
| |
| * History of any [[congenital]] disorders (e.g [[Down syndrome]], [[Bloom syndrome]])
| |
| |
| |
| * Bone tenderness
| |
| * Skin manifestations
| |
| |
| |
| * Immature Myeloblasts on blood smear
| |
| * Flow cytometry
| |
| * +Aur Rods
| |
| |-
| |
| |Hodgkin's, T- and B-cell lymphomas)
| |
| |
| |
| * Reed-Sternberg cell
| |
| ** B-cell origin
| |
| ** CD30 (Ki-1) and CD15 (Leu-M1) antigens
| |
| |
| |
| * Painless localized peripheral lymphadenopathy
| |
| * B symptoms
| |
| |
| |
| * Presence or absence, duration, and severity of other associated systemic symptoms.
| |
| * History of previous malignancy (including other lymphomas)
| |
| * Prior treatment with chemotherapy or radiotherapy
| |
| * Previous immunosuppressive illness
| |
| * Family history of HL or other lymphoproliferative, myeloproliferative, or tissue malignancies.
| |
| |
| |
| * Palpable, painless lymphadenopathy
| |
| * Superior vena cava
| |
| * Central nervous system (CNS) symptoms
| |
| * Paraneoplastic syndromes including
| |
| ** Cerebellar degeneration
| |
| ** Neuropathy
| |
| ** Guillain-Barre syndrome
| |
| ** Multifocal leukoencephalopathy
| |
| |
| |
| * Fine-needle aspiration
| |
| ** Mononucleate and binucleate Reed-Sternberg cells in a background of inflammatory cells
| |
| * Lactate dehydrogenase (LDH) may be increased.
| |
| * ESR elevated
| |
| * Serum creatinine elevated in nephrotic syndrome.
| |
| * Alkaline phosphatase (ALP) increased
| |
| * Hypercalcemia, hypernatremia, and hypoglycemia.
| |
| |-
| |
| |Churg-Strauss
| |
| (Eosinophilic granulomatosis with polyangiitis)
| |
| |
| |
| * Complex interaction involving [[Genetics|genetic]] and environmental factors that lead to an [[Inflammation|inflammatory response]] involving [[Eosinophil granulocyte|eosinophils]], [[B cell|lymphocytes]] and [[Macrophage|giant cells]]
| |
| |
| |
| * '''Prodromal phase:''' [[Rhinitis|Allergic rhinitis]] and [[asthma]].
| |
| * '''Eosinophilic phase:''' [[Eosinophilia|Peripheral eosinophilia]] and infiltration of [[Eosinophil granulocyte|eosinophils]] to [[lung]] and [[Gastrointestinal tract|GI tract]].
| |
| * '''Vasculitic phase:'''
| |
| ** Small and medium-sized [[vasculitis]] and inflammatory granuloma formation.
| |
| ** [[Granuloma|Granulomas]] can be either vascular or extravascular.
| |
| |
| |
| * History of allergy
| |
| *
| |
| |
| |
| * Skin involvement (60%)
| |
| * Nasal polyposis
| |
| * Peripheral neuropathy
| |
| |
| |
| * P-ANCA positive in most cases
| |
| * Elevated levels of [[Immunoglobulin E|IgE]]
| |
| * Elevated levels of [[rheumatoid factor]] at low titer
| |
| * [[Hypergammaglobulinemia]]
| |
| * Biopsy is diagnostic
| |
| ** Eosinophilic infiltration
| |
| ** Vasculitis of small and medium-sized vessels
| |
| ** [[Granuloma]] formation.
| |
| |-
| |
| |Systemic mastocytosis
| |
| |
| |
| * Infiltration of bone marrow by mast cell affecting the peripheral blood and coagulation system.
| |
| * The neoplastic clone of mast cells express abnormal cell surface markers CD25 and/or CD2.
| |
| |
| |
| * GI
| |
| * Cutaneous
| |
| * Urticaria pigmentosa
| |
| * Musculoskeletal
| |
| * Idiopathic and/or recurrent anaphylactoid reactions
| |
| |History of/ Associated with
| |
| * Hypereosinophilic syndrome
| |
| * Castleman disease
| |
| * Monoclonal gammopathy
| |
| * Hairy cell leukemia
| |
| * Non-Hodgkin lymphoma
| |
| * Polycythemia vera
| |
| * Primary thrombocythemia
| |
| |
| |
| * Signs of anemia,
| |
| * Hepatoslenomegaly
| |
| * Lymphadenopathy
| |
| * Urticaria
| |
| ** Flushing
| |
| * Osteolysis
| |
| |
| |
| * CBC
| |
| ** Eosinophilia
| |
| ** Basophilia
| |
| ** Thrombocytosis
| |
| ** Monocytosis
| |
| * Total–to–beta-tryptase ratio greater than 20:1 is suggestive.
| |
| * CD117 positive and CD25 and/or CD2 positive.
| |
| * Abnormal mast cells.
| |
| ** Larger than normal mast cells
| |
| ** Irregularly shaped nuclear outlines
| |
| ** Less densely packed mast cell granules
| |
| |}
| |
| <references /> | | <references /> |
|
| |
|