B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Burkitt's lymphoma: A case report and review

Abstract

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classical Burkitt's lymphoma (BL), is a diagnostic provisional category in the World Health Organization 2008 classification of lymphomas. This category was designed as a measure to accommodate borderline cases that cannot be reliably classified into a single distinct disease entity after all available morphological, immunophenotypical and molecular studies have been performed. Typically, these cases share features intermediate between DLBCL and classical BL or include characteristics of both lymphomas. The rarity of such cases poses a tremendous challenge to both pathologists and oncologists because its differential diagnosis has direct implications for management strategies. In this article, we present a “classical unclassifiable lymphoma with features intermediate between DLBCL and BL” in a young male patient and review of literature.

Comparison of Ki-67 Labeling Index Patterns of Diffuse Large B-Cell Lymphomas and Burkitt Lymphomas Using Image Analysis: A Multicenter Study

“…The majority of these cases possess translocations of MYC, BCL2 , and/or BCL6 ; these are called “double hit” or “triple hit” lymphomas and sometimes exhibit a blastoid appearance [ 1 , 3 , 10 , 11 , 12 , 13 ]. These cases were referred to in the 2008 World Health Organization classification as B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL, and were divided into two categories based on genetic abnormalities in the 2018 classification: HGBL with double/triple hit mutations (HGBL, DH/TH), and HGBL, not otherwise specified ( Supplementary Figure S1 ) [ 10 , 14 , 15 ].…”

Treatment of grey zone lymphoma using the R-CODOX-M/R-IVAC protocol

“…[ 1 ] This entity includes lymphomas that are characterized by clinical, morphological, and immunophenotypic features that overlap with those of DLBCL, such as primary mediastinal large B-cell lymphoma (PMBL) and nodular sclerosis CHL. [ 2 ] Although DLBCL, nodular sclerosis CHL, and GZL are closely related, these are distinct diseases. [ 3 ] Although extramediastinal site was found in several studies, these lymphomas commonly present as mediastinal lesion with high aggressiveness.…”

“…Immunohistochemically, the neoplastic cells show a blended phenotype of B-cell lymphoma (CD20, CD79a, PAX5, and OCT2) and CHL (CD15 and CD30). [ 2 , 6 ]…”

“…However, several studies have reported that GZL may also present at extramediastinal sites. [ 2 , 6 – 8 ] Owing to the relative rarity of these lymphomas, a consensus on the appropriate therapeutic approach is yet to be achieved. Current treatment approach is based on doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), while that for CHL and DLBCL requires addition of rituximab, based on CD20 expression of the neoplastic cells.…”