Diffuse large B-cell lymphoma with concomitant c-MYC and BCL6 gene rearrangements with primary skin involvement: A case report and a review of literature

Abstract

Double-hit lymphoma (DHL) is a rare aggressive B-cell lymphoma with concomitant c-MYC, BCL2 or BCL6 gene rearrangements, which is characterized by the high frequency of extranodal lesions and by resistance to chemotherapy. The median survival does not exceed 18 months in patients with this disease. The majority of DHL is represented by с-MYC/BCL2 cases. The combination of c-MYC/BCL6 occurs rarely (5-8%). The paper describes a case of DHL with concomitant c-MYC and BCL6 gene rearrangements, which mimics diffuse large B-cell lymphoma, leg-type.

Clinical manifestations of diffuse large B‑cell lymphoma in the skin and subcutaneous tissue a case series study

“…In a small percentage of cases these features may coexist, e.g. BCL2 and c-Myc (“double hit”), or additionally with BCL6 (“triple hit”) [ 11 ]. These subtypes are commonly associated with extranodal lesions and resistance to treatment [ 12 ].…”