套细胞淋巴瘤诊疗进展

Advances in the diagnosis and treatment of mantle cell lymphoma

套细胞淋巴瘤(mantle cell lymphoma,MCL)是一种少见的B细胞非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL)类型,兼具有惰性淋巴瘤和侵袭性淋巴瘤的临床病理特点。目前MCL的临床治疗仍然是以全身化疗为主,含有阿糖胞苷的强诱导化疗,随后行自体造血干细胞移植(autologous stem cell transplantation,Auto-HSCT)巩固,可以显著延长生存期,但MCL仍然属于不可治愈的淋巴瘤类型。近年来,随着对MCL发病机制的深入研究,更多的新药如Btk抑制剂、PI3K抑制剂、免疫调节剂等在MCL中得到应用。初治、复发难治MCL都具有更多的治疗选择,如何将新药和现有的化疗更为有机的结合,更好的改善MCL患者的生存期是未来研究的重点。

Mantle cell lymphoma （MCL） is a rare subtype of B-cell non-Hodgkin＇s lymphomas （NHLs）. MCL comprises distinct subtypes with different pathological characteristics and clinical features. However, MCL remains incurable by intensified first-line regimens con-taining cytarabine and involving consolidation with high-dose therapy and autologous stem cell transplantation. Recently discovered somatic mutations and aberrant intracellular signaling pathways have been demonstrated to play an essential role in the pathogenesis of MCL. The related novel therapeutics, such as Btk inhibitors, PI3K inhibitors, and immune modulators, have exhibited promising ther-apeutic effects on untreated or even relapsed/refractory MCL. The development of an efficient combination therapy is urgently need-ed to improve the survival of MCL patients in the future.