Burkitt Lymphoma and Burkitt-like Lymphoma Differential Diagnoses
- Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD more...
Burkitt lymphoma (BL) must be distinguished from other primary abdominal tumors in childhood, including Wilms tumor, neuroblastoma, and peripheral neuroectodermal tumor. In the bone marrow, BL must be differentiated from B and T precursor and myeloid leukemias. Among peripheral B-cell lymphomas, the major difficulty is to differentiate BL from diffuse large B-cell lymphoma (DLBCL), lymphoblastic lymphoma, and blastic mantle cell lymphoma (MCL).
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."[3, 44] (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%.[45, 46]
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.[47, 48] 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.[47, 48] 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.[47, 48] Unfortunately, microarray analysis is not readily available in most clinical settings and remains a research tool.
Lymphoblastic and mantle cell lymphomas
Lymphoblastic lymphoma may be histologically similar to Burkitt lymphoma (BL), however, it is a T-cell lymphoma that expresses T-cell markers in addition to TdT, which are usually negative in BL cases. Similarly, mantle cell lymphoma cases can be easily distinguished from Burkitt lymphoma (BL) by immunohistochemistry and flow cytometry as they typically stain strongly for CD5 and cyclin D1, unlike BL.
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