Diffuse large B cell lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Diffuse large B cell lymphoma may be classified into 14 subtypes based on immunophenotyping/genetic testing.

Classification

Diffuse large B-cell lymphoma encompasses a biologically and clinically diverse set of diseases,[1] many of which cannot be separated from one another by well-defined and widely accepted criteria.

The World Health Organization (WHO) classification system defines more than a dozen subtypes, each of which can be differentiated based on the

  • Location of the tumor
  • Presence of other cells within the tumor (such as T cells)
  • Other illnesses related to diffuse large B-cell lymphoma.
  • One of these well-defined groupings of particular note is Primary mediastinal B-cell lymphoma, which arises within the thymus or mediastinal lymph nodes.

B-cell lymphoma, unclassifiable

  • Tumor may share many features intermediate between Diffuse large B-cell lymphoma and Burkitt lymphoma.
  • Tumor may share many features intermediate between Diffuse large B-cell lymphoma and Hodgkin’s lymphoma.

Diffuse large B-cell lymphoma, not otherwise specified

  • When a case of Diffuse large B-cell lymphoma does not conform to any of the well-defined subtypes, and is also not considered unclassifiable.
  • The majority of Diffuse large B-cell lymphoma cases fall into this category.
  • Much research has been devoted to separating this still-heterogeneous group; such distinctions are usually made along lines of

Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and NK/T-Cell Neoplasms


 
 
 
 
 
 
 
 
 
B-cell neoplasms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CD5+
 
 
 
 
 
 
 
CD5-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CCND1+
 
 
 
CCND1-
 
 
 
 
DLBCL
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pleomorphic MCL
 
 
 
DLBCL, NOS CD5+
 
CD10+
 
 
 
 
 
 
 
CD10-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BCL6+ IRF4/MUM1-
 
BCL6+ IRF4/MUM1+
 
BCL6- IRF4/MUM1+
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
Non-GCB
 
Post-GCB
 
 

Classification of large cells based on Immunophenotypic/Genetic testing [2]

  • Diffuse large B-cell lymphoma (DLBCL),NOS
  • T-cell/histocyte-rich large B-cell lymphoma (THRLBCL)
  • Primary DLBCL of the CNS
  • Primary cutaneous DLBCL, leg type
  • EBV-positive DLBCL of the elderly (EBV + DLBCL)
  • DLBCL associated with chronic inflammation
  • Lymphoid granulomatosis
  • Primary mediastinal (thymic) large B-cell Lymphoma (PMBL)
  • Intravascular large B-cell lymphoma
  • ALK-positive large B-cell lymphoma
  • Plasmablastic lymphoma
  • Large B-cell lymphoma arising in HHV8-assciated multicentric Castleman disease (LBCL in HHV8 + MCD)
  • Primary effusion lymphoma
  • B-cell lymphoma unclassifiable, intermediate between DLBCL(U-DLBCL) and classical Hodgkins lymphoma(CHL)
  • Mantle cell Lymphoma(MCL),pleomorphic variant

References

  1. Alizadeh, Ash A.; Eisen, Michael B.; Davis, R. Eric; Ma, Chi; Lossos, Izidore S.; Rosenwald, Andreas; Boldrick, Jennifer C.; Sabet, Hajeer; Tran, Truc; Yu, Xin; Powell, John I.; Yang, Liming; Marti, Gerald E.; Moore, Troy; Hudson, James; Lu, Lisheng; Lewis, David B.; Tibshirani, Robert; Sherlock, Gavin; Chan, Wing C.; Greiner, Timothy C.; Weisenburger, Dennis D.; Armitage, James O.; Warnke, Roger; Levy, Ronald; Wilson, Wyndham; Grever, Michael R.; Byrd, John C.; Botstein, David; et al. (2000). "Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling". Nature. 403 (6769): 503–11. Bibcode:2000Natur.403..503A. doi:10.1038/35000501. PMID 10676951. line feed character in |title= at position 78 (help)
  2. "Non-Hodgkin's Lymphomas (NCCN.org)" (PDF).


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