Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations
Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations
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DOI：
10.3324/haematol.2008.005355
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发表时间：
2009-07-01
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影响因子：
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通讯作者：
Miura, Ikuo
中科院分区：
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作者：
Tomita, Naoto;Tokunaka, Mami;Miura, Ikuo
具有18q21.3/bcl2和8q24/myc易位到免疫球蛋白(IG)基因的淋巴肿瘤是一种罕见的双重打击淋巴瘤/白血病，临床转归较差。设计与方法为了阐明这种淋巴瘤/白血病的临床病理特征，我们分析了27例经细胞遗传学证实的双重打击淋巴瘤/白血病。诊断为双发淋巴瘤/白血病时，结外受累25例(93%)，中枢神经系统受累15例(56%)。诊断为淋巴瘤/白血病双重打击后，27例患者的中位生存期仅为6个月，1年生存率仅为22%。包括7例三联反应淋巴瘤/白血病(3q27/bcl6易位的双联反应淋巴瘤/白血病)，这些患者的中位生存期距离诊断为双联反应的淋巴瘤/白血病仅4个月。恶性三重打击患者的生存时间短于其他20例淋巴瘤/白血病患者(p=0.02)。检测14例侵袭性B细胞淋巴瘤患者的MIB-1指数，MYC-IgH易位组(n=7)高于MYC-IGK/L组(n=7)(P=0.02)。MYC-IGK/L易位组(n=14)和MYC-IGK或MYC-IGL易位组(n=13)的总生存率无差异。结论双重打击淋巴瘤/白血病是一种罕见但独特的成熟性B细胞肿瘤，预后极差，其特点是结外受累频繁，伴有MYC易位伙伴之一的中枢神经系统进展。
BackgroundLymphoid neoplasm with 18q21.3/BCL2 and 8q24/MYC translocation to immunoglobulin (IG) genes as dual-hit lymphoma/leukemia is very rare and known to have a poor clinical outcome.Design and MethodsTo clarify the clinicopathological characteristics of this malignancy, we analyzed 27 cases of cytogenetically proven dual-hit lymphoma/leukemia.ResultsDual-hit lymphoma/leukemia was diagnosed at presentation in 22 cases and at relapse or disease progression in 5 cases. At the time of diagnosis of dual-hit lymphoma/leukemia, extranodal involvement was found in 25 cases (93%) and central nervous system involvement occurred in 15 cases (56%). The median survival and 1-year survival rate of the 27 cases were only 6 months and 22%, respectively, after diagnosis of the dual-hit lymphoma/leukemia. Seven cases of triple-hit lymphoma/leukemia (dual-hit lymphoma/leukemia with 3q27/BCL6 translocation) were included; the median survival of these patients was only 4 months from the diagnosis of the dual-hit lymphoma/leukemia. The duration of survival of the patients with a triple-hit malignancy was shorter than that of the other 20 cases of dual-hit lymphoma/leukemia (p=0.02). The translocation partner of MYC subdivided the dual-hit cases into two groups; 14 cases of IGH and 13 cases of IGK/L The MIB-1 index was investigated in 14 cases with aggressive B-cell lymphoma, and was higher in the group with MYC-IGH translocation (n=7) than in the MYC-IGK/L group (n=7) (p=0.02). Overall survival was not different between the MYC-IGK/L translocation group (n=14) and the MYC-IGK or MYC-IGL translocation group (n=13).ConclusionsDual-hit lymphoma/leukemia is a rare but distinct mature B-cell neoplasm with an extremely poor prognosis characterized by frequent extranodal involvement and central nervous system progression with either of the translocation partners of MYC.