Clinical features and prognosis of de novo diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC translocations

Clinical features and prognosis of de novo diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC translocations
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DOI:
10.1038/leu.2008.344
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发表时间:
2009-04-01
期刊:
影响因子:
11.4
通讯作者:
Hirano, M.
Hirano, M.
中科院分区:
医学1区
文献类型:
--
作者:
Niitsu, N.;Okamoto, M.;Hirano, M.

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弥漫性大B细胞淋巴瘤(DLBCL)同时具有t(14;18)和8 q24易位是罕见的。我们评估了同时携带t(14; 18)和8 q24易位的DLBCL患者的临床特征和预后。1998年至2007年,成人淋巴瘤治疗研究组(ALTSG)共治疗了1972例非霍奇金淋巴瘤患者。共发现19例双易位的原发DLBCL。在394例DLBCL患者中观察到19例双易位(10例男性和9例女性,中位年龄为61岁)。双易位在高乳酸脱氢酶水平、B症状、骨髓受累和晚期患者中更常见。免疫表型分析显示大多数病例为弥漫大B细胞淋巴瘤伴生发中心型。双易位患者的无进展生存率和总生存率显著低于其他易位患者。同时发生t(14; 18)和8 q24易位的DLBCL患者预后极差。即使患者对化疗有完全反应,他们随后也会遭受早期复发。在这项研究中,只有少数患者接受利妥昔单抗,其有效性无法评估。需要更多患者的未来研究。
Diffuse large B-cell lymphoma (DLBCL) having both t(14;18) and 8q24 translocations is rare. We evaluated the clinical characteristics and prognoses of patients with DLBCL carrying both t(14; 18) and 8q24 translocations. A total of 1972 patients with non-Hodgkin's lymphoma were treated in the Adult Lymphoma Treatment Study Group (ALTSG) from 1998 to 2007. Nineteen cases of de novo DLBCL with the dual translocation were identified. The dual translocation was observed in 19 of 394 patients with DLBCL (10 males and 9 females, with a median age of 61 years). The dual translocation was observed significantly more frequently among patients with high lactate dehydrogenase levels, B symptoms, bone marrow involvement and advanced stage. Immunophenotyping was performed and showed DLBCL with a germinal center type in the majority of cases. Progression-free survival and overall survival rates were significantly lower in patients with the dual translocation than in those with other translocation. DLBCL patients with concurrent t(14; 18) and 8q24 translocations have very poor prognosis. Even if patients had a complete response to chemotherapy, they subsequently suffered early relapse. In this study, only a few patients received rituximab, and its usefulness could not be assessed. Future studies with larger numbers of patients are required.