What is diffuse large cell lymphoma?

Updated: Jun 12, 2019
  • Author: Shipra Gandhi, MBBS; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Diffuse large cell lymphoma is the most common lymphoma, representing 31% of the non-Hodgkin lymphomas (NHLs), and it is rapidly fatal if untreated. Clinically, patients with this type of lymphoma usually present with advanced, often extranodal disease. Histologically, these lymphomas contain an equal number of small and large cells (see the images below). (See Prognosis, Presentation, and Workup.)

Under the International Working Formulation, with regard to the classification of intermediate-grade diffuse large cell lymphomas, approximately 79% of these lymphomas were of B-cell origin; 16%, of T-cell origin; and 5%, unclassifiable. Exceptional cases expressed both B-cell and T-cell markers. (See Etiology.)

Incorporation of the Revised European-American Lymphoma (REAL) classification system for lymphomas has been strongly encouraged. In addition to morphologic descriptions, this schema includes immunologic, cytogenetic, and molecular information to define distinct lymphoma entities. Currently, diffuse large B-cell lymphoma is designated under the REAL classification as classic diffuse large cell lymphoma of B-cell origin defined by the working formulation. Lymphomas of T-cell or NK-cell origin exhibit biologic and clinical features distinct from diffuse large B-cell lymphomas. (See Workup.)

Diffuse large cell lymphoma of the REAL classification combines the large cell and the immunoblastic categories of the working formulation. These lymphomas are currently considered a single group, because they behave similarly and, therefore, have similar prognoses.


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