Abstract
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.
摘要
有IGH@BCL2 和MYC重排的大B细胞淋巴瘤称为双重打击淋巴瘤 (DHL),临床上侵袭性强预后差。 一些大B细胞淋巴瘤有同时发生的MYC和BCL2异常,但不是二者均为易位。关于这类被归为非典型DHL的特点我们所知甚少。我们研究了40 例非典型DHL ,男性21 人,女性19 人,中位年龄60岁。 9例 (23%) 患者有B细胞非霍奇金淋巴瘤病史。 30 例弥漫大B细胞淋巴瘤 (DLBCL), 7例具有介于DLBCL 和Burkitt 淋巴瘤特点之间、未分类的B细胞淋巴瘤, 3 例DLBCL伴滤泡淋巴瘤。 CD10、BCL2和MYC 表达阳性率分别为28/39 (72%), 33/35 (94%), 和14/20 (70%) 。患者经标准化疗 (n=14) 或更激进化疗 (n=17)。我们比较了非典型DHL 组和76例DHL以及35 例无MYC 和BCL2 异常的DLBCL。非典型和典型DHL患者的临床病理特点和治疗相似。非典型双重打击淋巴瘤患者总的生存率与双重打击淋巴瘤的相似(P=0.47), 相比MYC和BCL2正常的DLBCL来说 (P=0.02)明显更恶。二者稍有区别,即非典型双重打击淋巴瘤更具备DLBCL 形态(P<0.01), 不常表达CD10 (P<0.01),患者血清乳酸脱氢酶升高不多见(P=0.01)。总之,这些结果支持将MYC/BCL2 DHL 谱系扩展到包括大B细胞淋巴瘤伴MYC和BCL2 异常而非伴共易位的情况。
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