原发性浆细胞白血病的诊断和治疗-tumor-肿瘤杂志.pdfVIP

218 　　肿瘤2012 年3 月第32 卷第3 期　TUM OR Vol. 32, March 2012 DOI: 10.3781/j .issn.1000-7431.2012 .03.013 Copyright© 2012 by TUM OR Review 综述 · 原发性浆细胞白血病的诊断和治疗 丁倩倩，陈勤奋 复旦大学附属华山医院血液科，上海　200040 [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。 [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 [中图分类号]　R733.7 [文献标志码]　A [文章编号]　1000-7431 (2012) 03-02 18-04 Diagnosis and treatment of primary plasma cell leukemia DING Qian-qian, CHEN Qin-fen Department of Hematology, Huashan Hospital, Fudan University, Shanghai 200040, China [A BST RA CT]　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 bortezo- mib 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. [KEY WORDS]　Plasma cell leukemia, primary; Immunophenotype; Cytogenetics; Bortezomib; Prognosis [ TUMOR , 2012, 32(03):218-221] [8] 浆细胞白血病 （plasma cell leukemia，PCL ）是一种罕 在尸检中被发现全身多脏器发生广泛的浆细胞浸润 。然而， 见的、具有高度侵袭性的浆细胞疾病，以不良预后和对常规 由于PPCL 的发病率甚低，因此多为个案或少数病例的报道， 治疗的低反应率为其特征，骨