【摘要】：原發(fā)漿細胞白血病(primary plasma cell leukemia,PPCL)屬于高度侵襲性的漿細胞疾病。與多發(fā)性骨髓瘤相比,PPCL有其獨特的細胞遺傳學及分子生物學特征�；�,新藥如硼替佐米,免疫調(diào)節(jié)劑及造血干細胞移植的聯(lián)合應用提高了PPCL患者的預后和生存,而不同的遺傳及基因表達譜亦與不同預后相關。處于臨床試驗階段的部分靶向藥物如CD38單克隆抗體和CD138-CAR-T等也為PPCL的治療提供了新的方向。本文就近年來PPCL的診斷和治療的最新進展作一綜述,討論的主要問題包括有PPCL的診斷,PPCL的臨床及生化特征,PPCL的治療及預后和PPCL新藥研究現(xiàn)狀等。
[Abstract]:Primary plasmacytic leukemia (primary plasma cell leukemia,PPCL) is a highly invasive plasmacytic disease. Compared with multiple myeloma, PPCL has its unique cytogenetic and molecular biological characteristics. The combination of chemotherapy, new drugs such as bortezomil, immunomodulator and hematopoietic stem cell transplantation improved the prognosis and survival of PPCL patients, and different genetic and gene expression profiles were also associated with different prognosis. Some targeted drugs, such as CD38 monoclonal antibody and CD138-CAR-T, also provide a new direction for the treatment of PPCL. This paper reviews the recent advances in the diagnosis and treatment of PPCL. The main issues discussed include the diagnosis of PPCL, the clinical and biochemical characteristics of PPCL, the treatment and prognosis of PPCL, and the current status of new PPCL drugs.
【作者單位】： 南京醫(yī)科大學附屬無錫市人民醫(yī)院血液科;南京醫(yī)科大學第一附屬醫(yī)院血液科;
【分類號】：R733.7

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