[伴有c-MYC和BCL6基因重排且原发皮肤受累的弥漫性大B细胞淋巴瘤：一例报告及文献复习]

[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].

作者信息

Gabeeva N G, Koroleva D A, Belyaeva A V, Chernova N G, Kuzmina L A, Sudarikov A B, Obukhova T N, Kovrigina A M, Zvonkov E E, Savchenko V G

机构信息

FGBU 'Gematologicheskij nauchnyj tsentr' Minzdrava Rossii, Moskva, Rossija.

出版信息

Ter Arkh. 2017;89(7):85-92. doi: 10.17116/terarkh201789785-92.

DOI:10.17116/terarkh201789785-92

PMID:28766546

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.

摘要

双打击淋巴瘤（DHL）是一种罕见的侵袭性B细胞淋巴瘤，伴有c-MYC、BCL2或BCL6基因重排，其特征为结外病变发生率高且对化疗耐药。该疾病患者的中位生存期不超过18个月。大多数DHL为c-MYC/BCL2病例。c-MYC/BCL6的组合很少见（5-8%）。本文描述了一例伴有c-MYC和BCL6基因重排的DHL病例，该病例酷似腿部型弥漫性大B细胞淋巴瘤。