How is diffuse large B-cell lymphoma (DLBCL) classified?

Updated: Aug 20, 2020
  • Author: Shipra Gandhi, MBBS; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Considerable progress has been made in NHL classification.In 1982, the National Cancer Institute introduced the International Working Formulation, a translation system for other, older classifications, including the Rappaport and the immunologically oriented Lukes-Collins and Kiel systems. The International Working Formulation provided a conceptual framework that groups lymphomas as low grade (indolent), intermediate grade, or high grade, with respect to their natural histories. [4]  Of intermediate-grade diffuse large cell lymphomas, approximately 79% were of B-cell origin; 16%, of T-cell origin; and 5%, unclassifiable. Exceptional cases expressed both B-cell and T-cell markers. 

In 1994, the International Lymphoma Study Group proposed the Revised European-American Lymphoma (REAL) classification schema. [5] It classifies NHLs as being derived from B or T/natural killer (NK) cells, and it includes disease entities that were not part of the orking formulation.

In addition to morphologic descriptions, the REAL schema included immunologic, cytogenetic, and molecular information to define distinct lymphoma entities. The REAL classification combined the large cell and the immunoblastic categories of diffuse large cell lymphoma. Diffuse large B-cell lymphoma (DLBCL) is designated under the REAL classification as classic diffuse large cell lymphoma of B-cell origin. Lymphomas of T-cell or NK-cell origin exhibit biologic and clinical features distinct from DLBCLs. (See Workup.)

Currently, the World Health Organization schema is used to classify DLBCLs (see Overview/Pathophysiology).


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