Burkitt Lymphoma and Atypical Burkitt or Burkitt-like Lymphoma: Should These be Treated as Different Diseases?

Abstract

Burkitt lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma with an aggressive clinical course. Since the advent of short, intensive, multiagent chemoimmunotherapy regimens, it has carried a favorable prognosis. BL has been rather well characterized, whereas the other lymphomas morphologically resembling it are more heterogeneous. The cases classified as atypical BL/Burkitt-like lymphoma by the 2001 World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissue were thought to represent a continuum between BL and diffuse large B-cell lymphoma (DLBCL). The optimal therapeutic strategy for this provisional entity was not definitively established. However, recent incorporation of molecular genetic data into the 2008 WHO Classification has allowed further refinements with significant therapeutic implications, including the designation of a new provisional entity, “B-cell lymphoma, unclassifiable, with features intermediate between BL and DLBCL.” This review presents a comprehensive overview of the previously designated provisional entity of atypical BL/BLL in conjunction with a detailed comparison with BL and DLBCL.

Clinical Significance of MYC Expression and/or “High-grade” Morphology in Non-Burkitt, Diffuse Aggressive B-cell Lymphomas

“…In contrast, aggressive lymphomas that display some but not all of the morphologic, phenotypic or genetic features of BL have long created diagnostic challenges. 3,6–9 Specifically, there is morphologic overlap between BL and diffuse large B-cell lymphoma (DLBCL), and the characteristic phenotypic profile (CD10 positive, BCL2 negative, Ki67>95%) and genotypic hallmark ( MYC translocation positive) of BL may also be seen in DLBCL. Historically, lymphomas with such overlapping features have been variously, and controversially, classified using a variety of designations including small, non-cleaved, non-Burkitt lymphoma, Burkitt-like lymphoma and/or atypical Burkitt lymphoma.…”

“…1–9 The 2008 revision to the WHO classification introduced a new category for B-cell lymphoma, unclassified, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (hereafter abbreviated as “BCLU”). 10 Morphologically, BCLU are relatively monomorphic with intermediate sized to large cells, often with a “starry sky” growth pattern.…”

Outcomes of adults with relapsed or refractory Burkitt and high‐grade B‐cell leukemia/lymphoma

“…High-grade B-cell leukemia/lymphoma (HGBCL) is a group of rapidly proliferative B-cell neoplasms that lacks some of the characteristic morphologic, immunophenotypic, or molecular cytogenetic findings of BL but have a similarly aggressive clinical course. [2] HGBCL was adopted by the 2016 revision of the World Health Organization (WHO) classification and replaced the term “B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and BL.”[3]…”

Primary Central Nervous System B Cell Lymphoma, Unclassifiable, in an Immunocompetent Young Male

“…In addition to MYC translocation they also show other cytogenetic abnormality [8]. Cases that morphologically resemble Burkitt's lymphoma and are positive for BCL2 are placed under the category of double hit lymphoma when BCL2 and MYC translocations are present [9]. A systematic approach which includes morphology, immunohistochemistry and molecular studies is recommended for proper diagnosis of these aggressive B cell lymphoma due to significant overlapping between diffuse large B cell lymphoma, B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma and Burkitt's lymphoma (DLBCL/BL) and Burkitt's lymphoma [8].…”