Immunophenotypic variations of Burkitt lymphoma

Burkitt lymphoma (BL) exhibits a characteristic immunophenotype that is positive for pan-B-cell antigens and CD10 and expresses clonal surface immunoglobulins (SIgs). We evaluated 35 BLs and identified atypical immunophenotypes in 4 including SIg light chain negativity in 4, negativity for B-cell antigens in 2, lack of CD10 in 1, and CD4 expression in 1. All 4 cases showed morphologic features characteristic of BL, and all were confirmed by cytogenetic analysis. The 4 BL cases included 1 girl, 2 men with HIV/AIDS, and a third man with a history of heart transplantation. Two patients died shortly after diagnosis; the other 2 completed hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone with highly active antiretroviral therapy and achieved complete remission. Our study indicates that an unusual immunophenotype is not uncommon in BL. If the immunophenotype is interpreted in isolation, the diagnosis could be missed. Recognizing the variability of immunophenotype is essential for establishing an accurate diagnosis of BL.