摘要目的 探讨伴有MYC、bcl-2和bcl-6基因重排的高级别B细胞淋巴瘤(HGBL)的临床和病理学特点.方法 应用双色分离探针MYC、bcl-2和bcl-6对2016年1月至2017年4月中国医学科学院 北京协和医学院血液病医院收集的158例B细胞淋巴瘤患者的石蜡组织切片进行荧光原位杂交(FISH)检测,并结合临床病理资料进行分析.结果 158例B细胞淋巴瘤中MYC、bcl-2和bcl-6基因重排的HGBL为3例,1例合并CCND1/IgH阳性.3例均为中老年男性,多部位受累,乳酸脱氢酶(LDH)水平升高,Ann Arbor分期均为Ⅳ期;2例行高强度化疗,未缓解,生存期分别为9个月和11个月;1例未治疗,正在随访中.3例具有不同形态谱系,初诊分别为淋巴母细胞淋巴瘤、弥漫性大B细胞淋巴瘤和套细胞淋巴瘤.免疫表型检测示2例为生发中心B细胞型,1例为非生发中心B细胞型,Ki-67染色示3例均具有较高的增殖指数(60%~90%).结论 伴有MYC、bcl-2和bcl-6基因重排的HGBL侵袭性强,以多器官受累为主,分期晚,疗效差.需要结合病理组织学和FISH检测确诊,并需要与多种淋巴瘤鉴别.其生存期短,需要早期诊断与强化治疗.

abstractsObjective To investigate the clinicopathologic features of patients with high-grade B-cell lymphomas(HGBL)that have rearrangements of MYC, bcl-6 and bcl-2. Methods One hundred and fifty-eight B-cell lymphomas patients from Institute of Hematology and Blood Diseases Hospital from January 2016 to April 2017 were detected by fluorescence in situ hybridization(FISH)with double color split-apart probes. Results Among 158 B-cell lymphomas, 3 cases with MYC, bcl-2 and bcl-6 rearrangements were identified, 1 of which also had CCND1/IgH translocation. All three patients were of older age, with poor prognostic parameters, multiple organs involvements, elevated LDH and advanced-tumor stage. Two of the three patients were treated with high-intensity chemotherapy and had no remission with an overall survival of 9 months and 11 months respectively. One patient had follow-up with no treatment. Histologically, all three cases showed a spectrum of morphologic features. Although initially categorized as lymphoblastic lymphoma,diffuse large lymphoma and mantle cell lymphoma respectively,two cases were associated with germinal center B-cell(GCB)immunophenotype and 1 case with non-GCB immunophenotype. They had a high proliferation index as assessed by immunostaining for Ki-67(60%-90%). Conclusions MYC+bcl-2+bcl-6+HGBL is an aggressive disease with multiple organ involvement, high serum LDH levels,advanced stage disease,poor prognosis and shorter patient survival. The diagnosis should be made by histopathology combined with FISH analysis. Its separation from other types of B cell large cell lymphoma is of clinical importance.