外周T细胞淋巴瘤的研究进展

Peripheral T-cell lymphoma,unspecified(or not otherwise specfied):a review

外周T细胞淋巴瘤(peripheral T-cell lymphomas,PTCL)为异质性血液学肿瘤组,起源于成熟的T细胞,约占全部成人非霍奇金淋巴瘤(NHLs)15%。现今的WHO分类包括9种临床病理学外周T细胞NHLs,其中非特异变异型(PTCL-U)最常见。在诊断时常处于进展期,最常呈侵袭性临床经过,需立即治疗。为更好理解其生物学特性和探索新的治疗方法,本文综述PTCLs(特别着重PTCL-U亚组)的病理学、临床特征和目前的治疗策略。

Peripheral T - cell lymphomas（PTCL） comprises a heterogeneous group of haematological tumours ,which originate from mature T -cells, and constitute less than 15% of all non - Hodgkin＇s lymphomas（NHLs） in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T- cell NHLs, being the hnspecified variant＇（ PTCL - U ）the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs,with special attention to the group of PTCL- U.