Blastic mantle cell lymphoma with a Burkitt translocation

MYC gene rearrangements are observed as a genetic change in the blastoid variant of mantle cell lymphoma (MCL). We present two patients, one had 30% atypical lymphocytes with Burkitt-like morphology in the peripheral blood and the other had 30% blasts in the bone marrow. Both had a CCND1/IGH and an MYC rearrangement by cytogenetics and FISH. The immunophenotype was CD5+[one was a weak positive], CD10+, CD20+, CCND1+, >90% Ki-67 proliferation fraction and CD23 negative. The diagnosis was blastic MCL with Burkitt features. These cases, along with the reported cases, distinguish an aggressive category of a blastic MCL with a poor prognosis that probably requires Burkitt therapy.