A new subtype of lymphoma:double-hit lymphoma with bcl-2 and myc translocation / 白血病·淋巴瘤

ABSTRACT

Double-hit lymphoma (DHL) is a kind of disease with features intermediated between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL),usually accompanied by myc gene breakpoint with other recurrent chromosomal breakpoint and mainly involving myc and bcl-2 translocation.The presentation of this disease is characterized by elevated serum lactate dehydrogenase levels,B symptoms,bone marrow involvement,advanced stage disease,extranodal involvement,and central nervous system involvement.Because its features are similar with DLBCL and BL,it's difficult to distinguish them by pathological diagnosis.At present,the differential diagnosis is mainly by chromosomal analysis (G-banding),FISH and immunohistochemistry.This subtype received a poor response to conventional chemotherapy for DLBCL,and has a poor prognosis.The median survival time is only 0.2-1.5 years.Currently,the main regimens include RCHOP,RICE,RCVD,methotrexate prophylaxis for central nervous system involvement,high-dose chemotherapy and bone marrow transplantation.