How is Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL) differentiated from DLBCL?

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

DLBCL

DLBCL typically has larger cells, but some Burkitt lymphoma (BL) cases might have centroblast-like (large) cells intermixed with the more common medium-sized monoclonal lymphocytes, resulting in some degree of a diagnostic dilemma, especially when considering that up to 15% of DLBCL cases might test positive for the c-myc translocation. Those borderline cases are categorized under Burkitt-like lymphoma (BLL). In the 2008 World Health Organization (WHO) classification system, these are referred to as "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma." [2, 45] (See Histologic Features.)

Features that rule out the diagnosis of Burkitt lymphoma (BL) include bcl-6 gene rearrangement (independent from bcl-6 nuclear staining), bcl-2 positivity, presence of t(14;18), and a Ki67 staining of less than 95%. [46, 47]

Researchers have been able to define a specific genetic signature for Burkitt lymphoma (BL). Two different studies explored gene-expression profiling by microarray technology as a tool to accurately diagnose Burkitt lymphoma (BL) and differentiate it from DLBCL due to the significant difference in prognosis and treatment approaches for these diseases. [48, 49] Results showed that 17-34% of DLBCL cases diagnosed by expert pathologists had the typical Burkitt lymphoma (BL) genetic signature; likewise up to 4% of cases signed out as classic or atypical BL lacked the typical genetic signature.

In both studies, rare cases with the Burkitt signature were c-myc negative. [48, 49] Moreover, patients with a Burkitt lymphoma (BL) signature who were treated with CHOP-like (cyclophosphamide, hydroxydaunorubicin hydrochloride [doxorubicin hydrochloride], vincristine and prednisone) regimens had a worse prognosis than those patients who were treated with more intense chemotherapy regimens. [48, 49] Unfortunately, microarray analysis is not readily available in most clinical settings and remains a research tool.


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