B-cell lymphomas with MYC/8q24 rearrangements and IGH@BCL2/t(14;18) (q32;q21): an aggressive disease with heterogeneous histology, germina center B-cell immunophenotype and poor outcome
B-cell lymphomas with MYC/8q24 rearrangements and IGH@BCL2/t(14;18) (q32;q21): an aggressive disease with heterogeneous histology, germina center B-cell immunophenotype and poor outcome
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DOI：
10.1038/modpathol.2011.147
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发表时间：
2012-01-01
期刊：
影响因子：
7.5
通讯作者：
Medeiros, L. Jeffrey
中科院分区：
文献类型：
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作者：
Li, Shaoying;Lin, Pei;Medeiros, L. Jeffrey
伴有MYC/8q24重排和IGH@BCL2/t(14;18)（q32;q21）的b细胞淋巴瘤，也被称为双重打击或MYC/BCL2 b细胞淋巴瘤，是一种罕见的肿瘤。我们报告了60例病例的经验：52例MYC/BCL2 b细胞淋巴瘤和8例肿瘤伴有额外的MYC信号+ IGH@BCL2或MYC重排+额外的BCL2信号/拷贝。有38名男性和22名女性，中位年龄为55岁。总共有10例患者有先发/并发滤泡性淋巴瘤。根据2008年世界卫生组织的分类，有33例b细胞淋巴瘤无法分类，其特征介于弥漫性大b细胞淋巴瘤和伯基特淋巴瘤之间（以下称为无法分类的侵袭性b细胞淋巴瘤），23例弥漫性大b细胞淋巴瘤，1例滤泡性淋巴瘤38级，1例滤泡性淋巴瘤合并弥漫性大b细胞淋巴瘤，1例b淋巴母细胞淋巴瘤，1例混合性弥漫性大b细胞淋巴瘤合并b淋巴母细胞淋巴瘤。使用较旧的分类系统，33例无法分类的侵袭性b细胞淋巴瘤与伯基特样淋巴瘤（n=24）或BCL2表达的非典型伯基特淋巴瘤（n=9）最为相似。在评估的48例病例中，47例（98%）具有生发中心b细胞免疫表型。患者接受标准（n=23）或更积极的化疗方案（n=34）。对57例患者进行了充分的随访：26例死亡，31例存活。对于52例MYC/BCL2淋巴瘤患者，中位总生存期为18.6个月。既往/并发滤泡性淋巴瘤患者的中位总生存期为7.8个月。血清乳酸脱氢酶水平升高，结外部位>= 2，骨髓或中枢神经系统受累，以及国际预后指数>2与总生存期较差相关(P
B-cell lymphomas with MYC/8q24 rearrangement and IGH@BCL2/t(14;18)(q32;q21), also known as double-hit or MYC/BCL2 B-cell lymphomas, are uncommon neoplasms. We report our experience with 60 cases: 52 MYC/BCL2 B-cell lymphomas and 8 tumors with extra MYC signals plus IGH@BCL2 or MYC rearrangement plus extra BCL2 signals/copies. There were 38 men and 22 women with a median age of 55 years. In all, 10 patients had antecedent/concurrent follicular lymphoma. Using the 2008 World Health Organization classification, there were 33 B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (henceforth referred to as unclassifiable, aggressive B-cell lymphoma), 23 diffuse large B-cell lymphoma, 1 follicular lymphoma grade 38, 1 follicular lymphoma plus diffuse large B-cell lymphoma, 1 B-lymphoblastic lymphoma, and 1 composite diffuse large B-cell lymphoma with B-lymphoblastic lymphoma. Using older classification systems, the 33 unclassifiable, aggressive B-cell lymphomas most closely resembled Burkitt-like lymphoma (n=24) or atypical Burkitt lymphoma with BCL2 expression (n=9). Of 48 cases assessed, 47 (98%) had a germinal center B-cell immunophenotype. Patients were treated with standard (n=23) or more aggressive chemotherapy regimens (n=34). Adequate follow-up was available for 57 patients: 26 died and 31 were alive. For the 52 patients with MYC/BCL2 lymphoma, the median overall survival was 18.6 months. Patients with antecedent/concurrent follicular lymphoma had median overall survival of 7.8 months. Elevated serum lactate dehydrogenase level, >= 2 extranodal sites, bone marrow or central nervous system involvement, and International Prognostic Index >2 were associated with worse overall survival (P