原发性浆细胞白血病的诊断和治疗  

Diagnosis and treatment of primary plasma cell leukemia

作　　者：丁倩倩[1] 陈勤奋[1] 

机构地区：[1]复旦大学附属华山医院血液科,上海200040

出　　处：《肿瘤》2012年第3期218-221,共4页Tumor

摘　　要：原发性浆细胞白血病(primary plasma cell leukemia,PPCL)是一种罕见的、具有高度侵袭性的浆细胞疾病,对常规治疗反应率低且预后不良。PPCL发病前无浆细胞骨髓瘤病史。近年来,随着新型药物如硼替佐米以及新治疗方法如造血干细胞移植的应用,PPCL患者的预后和生存得到了改善。本文对PPCL诊断和治疗的最新进展进行综述,以期为临床实践提供有益的借鉴。Primary plasma cell leukemia（PPCL） is a rare,highly aggressive plasma cell disease characterized with a poor prognosis and a low response rate to conventional therapy.It occurs de novo in the absence of a prior history of plasma cell myeloma.Recently,the use of novel drugs such as bortezomib and the approaches such as stem cell transplantation may improve the outcomes of PPCL.This review summarizes the diagnosis and treatment of PPCL in order to provide reference information for clinical practice.

关 键 词：浆细胞白血病 原发性 免疫表型 细胞遗传学 硼替佐米 预后 

分 类 号：R733.7[医药卫生—肿瘤]