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==Overview==
==Overview==

Revision as of 16:40, 2 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2] Anila Hussain, MD [3]

Overview

Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on molecular, genetic, and immunohistochemical features into more than 20 subgroups.

Classification

Classification Based on Location

Diffuse large B cell lymphoma may be classified based on location:

  • Nodal disease
  • Extranodal disease

Classification Based on Molecular, Genetic, and Immunohistochemical Features

According to the WHO classification, diffuse large B cell lymphoma may be classified based on molecular, genetic, and immunohistochemical features into the following:[1]

1. Diffuse large B cell lymphoma, not otherwise specified

A- Morphologic subgroups
  • Centroblastic Variant
  • Most common variant, 80 percent of all cases
  • Appearance of medium-to-large-sized lymphocytes
  • Tumor may be monomorphic, composed almost entirely of Centroblasts(>90%)
  • The majority of cases are polymorphic (mixture of Centroblasts(<90%), Immunoblasts and Centrocytes)
  • Immunoblastic Variant
  • 8-10 percent of all cases of DLBCL
  • Greater than 90% of its cells are immunoblasts
    • Large lymphoid cells with Significant basophilic cytoplasm
    • Trapezoid shaped centrally located nucleolus with fine chromatin strands that are attached to nuclear membrane(also known as spider legs)
  • Anaplastic Variant
  • Less common variant comprising almost 3 percent of all cases of DLBCL
  • Tumor cells which appear very differently from their normal B cell counterparts
    • Very large cells with a round, oval, or polygonal shape that may resemble Reed-Sternberg cells of Hodgkin's lymphoma or Anaplastic Large cell Lymphoma
    • Pleomorphic nuclei
  • Sinusoidal Pattern
  • Other
  • Does not meet any of the above criteria
B- Immunophenotypic subgroups
  • Germinal center-derived B-cell (GCB)
  • Activated B-cell-like (ABC/Non GCB)
C- Genetic subgroups
  • BCL6
  • BCL2
  • C-MYC
  • Other
D- Immunohistochemical subgroups
  • CD5-positive de-novo diffuse large B cell lymphoma
  • Germinal center B-cell like (GCB)
  • Non-germinal center B-cell-like (non-GCB)

2. Diffuse Large B Cell Lymphoma(DLBCL) subtypes

  • T-cell/histiocyte-rich large B cell lymphoma
  • Primary diffuse large B cell lymphoma of the central nervous system (CNS)
  • Epstein-Barr virus positive diffuse large B cell lymphoma, Not specified Otherwise
  • Primary Cutaneous DLBCL, Leg type

3. Other lymphomas of large B cells

  • Primary mediastinal (thymic) large B-cell lymphoma
  • Intravascular large B-cell lymphoma
  • DLBCL associated with chronic inflammation
  • Lymphomatoid granulomatosis
  • ALK-positive LBCL
  • Plasmablastic lymphoma
  • Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
  • Primary effusion lymphoma

4. Borderline cases B-cell lymphoma

  • High Grade B cell Lymphoma with MYC and BCL6 And/Or BCL4 Translocations
  • High Grade B cell Lymphoma, Not Otherwise Specified
  • B cell Lymphoma, Unclassifiable with features intermixed with DLBCL and Classical Hodgkin Lymphoma

References

  1. Stein H, Chan JKC, Warnke RA (2008). Diffuse large B-cell lymphoma not otherwise specified. In:Swerdlow SH, Campo E, Harris NL, et al. editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC. p. 233-7.


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