Non-Hodgkin lymphoma pathophysiology: Difference between revisions
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! rowspan="2" |Pathophysiology | ! rowspan="2" |Pathophysiology | ||
! rowspan="2" |Symptoms | ! rowspan="2" |Symptoms | ||
! rowspan="2" |Physical Examination | ! rowspan="2" |Physical Examination | ||
! colspan="3" |Laboratory Findings | ! colspan="3" |Laboratory Findings | ||
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|[[Diffuse large B-cell lymphoma]] | |[[Diffuse large B-cell lymphoma]] | ||
|Classified into 2 subtypes based on [[gene expression]] profiles: | |Classified into 2 subtypes based on [[gene expression]] profiles: | ||
*Germinal centre B-cell-like (GCB) | *Germinal centre B-cell-like (GCB) | ||
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* | * | ||
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* Non tender mass | * Non tender mass | ||
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|[[Follicular lymphoma]] | |[[Follicular lymphoma]] | ||
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* Reciprocal translocation t(14;18)(q32;q21). | * Reciprocal translocation t(14;18)(q32;q21). | ||
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*[[Dyspnea]] | *[[Dyspnea]] | ||
*Painless swelling in the neck, axilla, groin, thorax and abdomen | *Painless swelling in the neck, axilla, groin, thorax and abdomen | ||
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* [[Lymphadenopathy|Central and peripheral lymphadenopathy]] | * [[Lymphadenopathy|Central and peripheral lymphadenopathy]] | ||
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* Unexplained [[weight loss]] | * Unexplained [[weight loss]] | ||
* [[Swollen lymph nodes]] in the neck, axilla, or groin | * [[Swollen lymph nodes]] in the neck, axilla, or groin | ||
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* [[Proptosis]] | * [[Proptosis]] | ||
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* Abdominal distention | * Abdominal distention | ||
* Fatigue | * Fatigue | ||
* Night sweats | |||
* Weight Loss | |||
* Extranodal involvement of gastrointtestinal (GI) tract, lungs, and central nervous system (CNS) | * Extranodal involvement of gastrointtestinal (GI) tract, lungs, and central nervous system (CNS) | ||
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* Generalized lymphadenopathy | * Generalized lymphadenopathy | ||
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** Abdominal pain | ** Abdominal pain | ||
** Hemorrhage | ** Hemorrhage | ||
* Chronic infectious conditions or autoimmune processes, such as | * Chronic infectious conditions or autoimmune processes, such as | ||
** ''H pylori'' gastritis | ** ''H pylori'' gastritis | ||
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** Deletion 7q21-32 | ** Deletion 7q21-32 | ||
** Translocations of the CDK6 gene located at 7q21. | ** Translocations of the CDK6 gene located at 7q21. | ||
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* Recurrent [[Infection|infections]] | * Recurrent [[Infection|infections]] | ||
* Early satiety | * Early satiety | ||
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* [[Pallor]], | * [[Pallor]], | ||
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|[[Multiple myeloma|Plasma cell myeloma]] | |[[Multiple myeloma|Plasma cell myeloma]] | ||
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|[[Chronic lymphocytic leukemia]] / [[Chronic lymphocytic leukemia|small lymphocytic lymphoma]] | |[[Chronic lymphocytic leukemia]] / [[Chronic lymphocytic leukemia|small lymphocytic lymphoma]] | ||
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|[[Monoclonal B-cell lymphocytosis]] | |[[Monoclonal B-cell lymphocytosis]] | ||
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|[[B-cell prolymphocytic leukemia]] | |[[B-cell prolymphocytic leukemia]] | ||
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|[[Waldenström's macroglobulinemia|Waldenström's macroglubulinemia]] | |[[Waldenström's macroglobulinemia|Waldenström's macroglubulinemia]] | ||
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|[[Monoclonal gammopathy of undetermined significance]] (MGUS) | |[[Monoclonal gammopathy of undetermined significance]] (MGUS) | ||
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|[[Heavy chain disease]] | |[[Heavy chain disease]] | ||
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|Solitary plasmacytoma of bone | |Solitary plasmacytoma of bone | ||
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|Extraosseous plasmacytoma | |Extraosseous plasmacytoma | ||
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|Monoclonal immunoglobulin deposition diseases | |Monoclonal immunoglobulin deposition diseases | ||
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|Extranodal marginal zone lymphoma of [[MALT lymphoma|mucosa-associated lymphoid tissue]] (MALT lymphoma) | |Extranodal marginal zone lymphoma of [[MALT lymphoma|mucosa-associated lymphoid tissue]] (MALT lymphoma) | ||
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|Large B-cell lymphoma with IRF4 rearrangement | |Large B-cell lymphoma with IRF4 rearrangement | ||
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|[[Primary cutaneous follicle centre lymphoma|Primary cutaneous follicle center lymphoma]] | |[[Primary cutaneous follicle centre lymphoma|Primary cutaneous follicle center lymphoma]] | ||
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|T-cell/histiocyte-rich large B-cell lymphoma | |T-cell/histiocyte-rich large B-cell lymphoma | ||
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|[[Lymphomatoid granulomatosis]] | |[[Lymphomatoid granulomatosis]] | ||
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|[[Primary mediastinal large B-cell lymphoma|Primary mediastinal (thymic) large B-cell lymphoma]] | |[[Primary mediastinal large B-cell lymphoma|Primary mediastinal (thymic) large B-cell lymphoma]] | ||
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|[[Intravascular large B-cell lymphoma]] | |[[Intravascular large B-cell lymphoma]] | ||
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|ALK1 large B-cell lymphoma | |ALK1 large B-cell lymphoma | ||
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|Plasmablastic lymphoma | |Plasmablastic lymphoma | ||
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|[[Primary effusion lymphoma]] | |[[Primary effusion lymphoma]] | ||
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|High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements | |High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements | ||
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* [[Anemia]] | * [[Anemia]] | ||
* May progress to [[Sezary syndrome]] (Skin involvement plus hematogenous dissemination) | * May progress to [[Sezary syndrome]] (Skin involvement plus hematogenous dissemination) | ||
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* Cutaneous manifestaions | * Cutaneous manifestaions | ||
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* [[Bone pain]] | * [[Bone pain]] | ||
* [[Dyspnea]] | * [[Dyspnea]] | ||
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* Usually appear pale and malnourished. | * Usually appear pale and malnourished. | ||
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|[[Subcutaneous panniculitis-like T-cell lymphoma]] | |[[Subcutaneous panniculitis-like T-cell lymphoma]] | ||
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Revision as of 15:06, 27 December 2018
Non-Hodgkin lymphoma Microchapters |
Differentiating Non-Hodgkin's Lymphoma |
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Non-Hodgkin lymphoma pathophysiology On the Web |
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Risk calculators and risk factors for Non-Hodgkin lymphoma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Non Hodgkin's Lymphoma represents a heterogeneous group of diseases with varied clinical presentation and histological appearance.It arises from cell of the lymphoid system, tumors are mainly derived from B lymphocytes, but are also from T lymphocytes, or natural killer cells. Lymphomas rise from different stages of B and T cell differentiation. Aberrations in the tightly controlled steps of B cell development can lead to oncogenesis. These aberrations are mainly seen in form of chromosomal translocation.
Pathophysiology
Pathogenesis
- Lymphomas can arise from different stages of B cell development:
- B cell development starts in the primary lymphoid tissue, the bone marrow and subsequent maturation takes place in secondary lymphoid tissue (spleen and lymph nodes).
- At the germinal centers of secondary lymphoid tissue B cells encounter antigens via T cells and then undergo affinity maturation to produce immunoglobulins of high affinity.
- It supports rapid B-cell proliferation for immunoglobulin affinity maturation and production of antibody diversity through two processes know as somatic hypermutation (SHM) and immunoglobulin class switching.
- Both of these processes require rapid cell turnover and multiple double stranded DNA breaks, which is error-prone.
- Somatically acquired genetic alterations ( mainly translocations) of these processes is probably the underlying cause of lymphomagenesis.
- The major subtypes of non-hodgkin lymphoma include the following:
- Mature B-cell neoplasms:
- Diffuse large B cell lymphoma
- Follicular lymphoma
- Burkitt lymphoma
- Mantle cell lymphoma
- Hairy cell leukemia
- Extranodal marginal zone lymphoma
- Splenic marginal zone lymphoma
- Plasma cell myeloma
- Mature T and NK neoplasms:
- Mature B-cell neoplasms:
Genetics
Different subtypes of non Hodgkin lymphoma and their genetic involvements::
Pathophysiology | Symptoms | Physical Examination | Laboratory Findings | ||||
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Immunochemistry | Blood work | Biospy | |||||
Diffuse large B-cell lymphoma | Classified into 2 subtypes based on gene expression profiles:
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Centroblastic
Immunoblastic::
Anaplastic:
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Follicular lymphoma |
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Burkitt lymphoma |
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B cell lymphoma | Mantle cell lymphoma |
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CBC
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Nodal marginal zone B-cell lymphoma |
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Splenic marginal zone lymphoma |
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Hairy cell leukemia |
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Plasma cell myeloma | |||||||
Chronic lymphocytic leukemia / small lymphocytic lymphoma | |||||||
Monoclonal B-cell lymphocytosis | |||||||
B-cell prolymphocytic leukemia | |||||||
Waldenström's macroglubulinemia | |||||||
Monoclonal gammopathy of undetermined significance (MGUS) | |||||||
Heavy chain disease | |||||||
Solitary plasmacytoma of bone | |||||||
Extraosseous plasmacytoma | |||||||
Monoclonal immunoglobulin deposition diseases | |||||||
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) | |||||||
Large B-cell lymphoma with IRF4 rearrangement | |||||||
Primary cutaneous follicle center lymphoma | |||||||
T-cell/histiocyte-rich large B-cell lymphoma | |||||||
Lymphomatoid granulomatosis | |||||||
Primary mediastinal (thymic) large B-cell lymphoma | |||||||
Intravascular large B-cell lymphoma | |||||||
ALK1 large B-cell lymphoma | |||||||
Plasmablastic lymphoma | |||||||
Primary effusion lymphoma | |||||||
High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements | |||||||
T cell lymphoma | Mycosis fungoides / Sézary syndrome |
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T-cell granular lymphocytic leukemia |
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Symptoms of T-cell large granular lymphocyte leukemia may include the following: |
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Subcutaneous panniculitis-like T-cell lymphoma |
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
Genetics
The development of Non-Hodgkin lymphoma is the result of multiple genetic mutations such as:[1][2]
- Mutations of the B-cell receptor genes and NFKB pathway
- RNA splicing mutations in the small lymphocytic lymphoma
- Genetic mutations in histone formation:[3]
- MLL2
- MEF2B
- EZH2
- CREBBP
- EP300
- MLL2
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ Pasqualucci L, Trifonov V, Fabbri G, Ma J, Rossi D, Chiarenza A; et al. (2011). "Analysis of the coding genome of diffuse large B-cell lymphoma". Nat Genet. 43 (9): 830–7. doi:10.1038/ng.892. PMC 3297422. PMID 21804550.
- ↑ Lohr JG, Stojanov P, Lawrence MS, Auclair D, Chapuy B, Sougnez C; et al. (2012). "Discovery and prioritization of somatic mutations in diffuse large B-cell lymphoma (DLBCL) by whole-exome sequencing". Proc Natl Acad Sci U S A. 109 (10): 3879–84. doi:10.1073/pnas.1121343109. PMC 3309757. PMID 22343534.
- ↑ Green MR, Gentles AJ, Nair RV, Irish JM, Kihira S, Liu CL; et al. (2013). "Hierarchy in somatic mutations arising during genomic evolution and progression of follicular lymphoma". Blood. 121 (9): 1604–11. doi:10.1182/blood-2012-09-457283. PMC 3587323. PMID 23297126.