Division of Hematopathology, Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Vanderbilt University School of Medicine, Nashville, TN, USA.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Mod Pathol. 2015 Feb;28(2):208-17. doi: 10.1038/modpathol.2014.95. Epub 2014 Aug 8.
Large B-cell lymphomas with IGH@BCL2 and MYC rearrangement, known as double-hit lymphoma (DHL), are clinically aggressive neoplasms with a poor prognosis. Some large B-cell lymphomas have concurrent abnormalities of MYC and BCL2 other than coexistent translocations. Little is known about patients with these lymphomas designated here as atypical DHL. We studied 40 patients of atypical DHL including 21 men and 19 women, with a median age of 60 years. Nine (23%) patients had a history of B-cell non-Hodgkin lymphoma. There were 30 diffuse large B-cell lymphoma (DLBCL), 7 B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma, and 3 DLBCL with coexistent follicular lymphoma. CD10, BCL2, and MYC were expressed in 28/39 (72%), 33/35 (94%), and 14/20 (70%) cases, respectively. Patients were treated with standard (n=14) or more aggressive chemotherapy regimens (n=17). We compared the atypical DHL group with 76 patients with DHLand 35 patients with DLBCL lacking MYC and BCL2 abnormalities. The clinicopathologic features and therapies were similar between patients with atypical and typical DHL. The overall survival of patients with atypical double-hit lymphoma was similar to that of patients with double-hit lymphoma (P=0.47) and significantly worse than that of patients with DLBCL with normal MYC and BCL2 (P=0.02). There were some minor differences. Cases of atypical double-hit lymphoma more often have DLBCL morphology (P<0.01), less frequently expressed CD10 (P<0.01), and patients less often had an elevated serum lactate dehydrogenase level (P=0.01). In aggregate, these results support expanding the category of MYC/BCL2 DHL to include large B-cell lymphomas with coexistent MYC and BCL2 abnormalities other than concurrent translocations.
具有IGH@BCL2 和 MYC 重排的大 B 细胞淋巴瘤，称为双打击淋巴瘤（DHL），是临床侵袭性强、预后不良的肿瘤。一些大 B 细胞淋巴瘤除了共存易位外，还存在 MYC 和 BCL2 的并发异常。对于这些被指定为非典型 DHL 的淋巴瘤患者，我们知之甚少。我们研究了 40 例非典型 DHL 患者，包括 21 名男性和 19 名女性，中位年龄为 60 岁。9 例（23%）患者有 B 细胞非霍奇金淋巴瘤病史。其中 30 例为弥漫性大 B 细胞淋巴瘤（DLBCL），7 例为 B 细胞淋巴瘤，无法分类，介于 DLBCL 和伯基特淋巴瘤之间，3 例为 DLBCL 合并滤泡性淋巴瘤。CD10、BCL2 和 MYC 的表达率分别为 28/39（72%）、33/35（94%）和 14/20（70%）。患者接受标准（n=14）或更具侵袭性的化疗方案（n=17）治疗。我们将非典型 DHL 组与 76 例 DHL 患者和 35 例缺乏 MYC 和 BCL2 异常的 DLBCL 患者进行比较。非典型和典型 DHL 患者的临床病理特征和治疗方法相似。非典型双打击淋巴瘤患者的总生存率与双打击淋巴瘤患者相似（P=0.47），明显低于 MYC 和 BCL2 正常的 DLBCL 患者（P=0.02）。存在一些细微差异。非典型双打击淋巴瘤病例更常具有 DLBCL 形态（P<0.01），较少表达 CD10（P<0.01），且患者血清乳酸脱氢酶水平升高的情况较少（P=0.01）。总之，这些结果支持将 MYC/BCL2 DHL 的范畴扩大到包括共存 MYC 和 BCL2 异常的大 B 细胞淋巴瘤，而不仅仅是共存易位。
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