Non-Hodgkin lymphoma pathophysiology: Difference between revisions
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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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|[[Follicular lymphoma]] | |[[Follicular lymphoma]]<ref name="pmid12529293">{{cite journal |author=Bosga-Bouwer AG, van Imhoff GW, Boonstra R, ''et al.'' |title=Follicular lymphoma grade 3B includes 3 cytogenetically defined subgroups with primary t(14;18), 3q27, or other translocations: t(14;18) and 3q27 are mutually exclusive |journal=Blood |volume=101 |issue=3 |pages=1149–54 |date=February 2003 |pmid=12529293 |doi=10.1182/blood.V101.3.1149 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12529293}}</ref> | ||
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* Reciprocal translocation t(14;18)(q32;q21). | |||
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Revision as of 03:23, 27 December 2018
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Non-Hodgkin lymphoma Microchapters |
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Differentiating Non-Hodgkin's Lymphoma |
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Non-Hodgkin lymphoma pathophysiology On the Web |
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American Roentgen Ray Society Images of Non-Hodgkin lymphoma pathophysiology |
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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 | History | 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[1] |
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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 | ||||||||
- ↑ Bosga-Bouwer AG, van Imhoff GW, Boonstra R; et al. (February 2003). "Follicular lymphoma grade 3B includes 3 cytogenetically defined subgroups with primary t(14;18), 3q27, or other translocations: t(14;18) and 3q27 are mutually exclusive". Blood. 101 (3): 1149–54. doi:10.1182/blood.V101.3.1149. PMID 12529293.
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.