套细胞淋巴瘤的临床特征和治疗学进展

套细胞淋巴瘤(MCL)包括在WHO分类中具t(11;14)(q13;q23)易位的淋巴瘤,是以CyclinD1过度表达为特征的独立淋巴瘤亚型.临床表现常包括结外累及,尤其是骨髓和肠道.在B细胞淋巴瘤患者中预后最差,中位总生存期3～5年,常规化疗不能治愈,但常可获得60%～90%的缓解率,其缓解期比其他淋巴瘤为短(1～2年).很强烈的治疗(包括自体和异基因干细胞移植)为改善转归所必需,但对于诊断时中位年龄≥60岁的患者其可行性有限.预后因子前瞻性调查有助于对患者的诊断、治疗.

Clinical features and therapeutic progress in mantle cell lymphoma

Mantle cell lymphoma is included in the World Health Organization classification as distinct lymphoma subtype characterized by the t(11;14)(q13;q32) translocation,which results in overexpression of Cyclin D1.The clinical presentation often includes extranodal involvement,particularly of the bone marrow and gut.The prognosis of patients with mantle cell lymphoma (median overall survival,3-5 years) is poorest among B-cell lymphoma patients,even though a prospectively difficult to identify subgroup can survive for vears with little or no treatment. Conventional chemotherapy is not curative but obtains frequent remissions (60%-90%) which are usually shorter (1-2 years) compared with other lymphoma entities.Very intensive regimens,including autologous and allogeneic stem cell transplantation,seem required to improve the outcome,but with the median age of diagnosis being 60 years or more,such approaches are feasible only in a limited proportion of patients.The possibility of treating patients based on prognostic factors needs to be investigated prospectively.