Double-Hit Lymphoma Presenting as Primary Renal Lymphoma: A Case Report

Abstract

Vikas Mehta, Chandy Ellimottil, Jodi Speiser, Milind Velankar, G

B-cell lymphomas with concurrent MYC with BCL2 and/or BCL6 rearrangements, also known as “double hit” lymphomas (DHL), are rare neoplasms characterized by highly aggressive clinical behavior, complex karyotypes, and a spectrum of pathological features overlapping with Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL). Primary renal lymphoma (PRL) by definition is a renal lymphoma without evidence of systemic involvement. PRL is extremely rare with less than 100 cases of both Hodgkin disease and non-Hodgkin lymphoma reported in literature. Double hit lymphomas have extremely poor prognosis, and high resistance to intensive chemotherapy, including high-dose chemotherapy. We describe a very rare case of DHL arising in kidney as PRL in whom concurrent MYC and IGH-BCL2 rearrangements were detected.