【摘要】 目的:探讨原发性浆细胞白血病(PPCL)的临床特征与治疗,提高对该病的认识。方法:对本院1987年5月至2009年4月确诊的22例PPCL患者的临床资料进行回顾性分析。结果:22例患者中,男性13例,女性9例;年龄33～76岁,平均年龄(53.3±13.3)岁。主要临床特征为贫血者19例,乏力17例,骨痛15例,发热9例,出血9例,体重下降7例,肝肿大7例,脾肿大11例,淋巴结肿大7例,胸腔积液6例,腹腔积液4例,心包积液2例,盆腔积液2例,双下肢浮肿3例。实验室检查以外周血白细胞升高(68.2%)、贫血(86.4%)、血小板减少(86.4%)、M蛋白、肾功能不全、低蛋白血症、ESR增快和血清尿酸升高常见。骨质破坏常见。多种常规化疗方案均难以达到或维持长期临床缓解。10例予常规化疗方案,其中1例一过性CR、6例PR、3例进展;1例常规化疗联合沙利度胺,获得CR长达7个月;3例应用硼替佐米联合化疗或沙利度胺均获PR;2例因肺部感染、肝功能异常以及心功能不全,不能化疗,分别生存1、4个月;1例未开始治疗即死亡;5例放弃治疗。结论:PPCL在临床特点上兼有急性白血病与多发性骨髓瘤的特征,且更加倾向于急性白血病,预后极差。新药硼替佐米在PPCL的靶向治疗中具有积极显著的作用,为患者提供了新的治疗希望。硼替佐米联合化疗及沙利度胺有望提高对PPCL的疗效。更多还原

【Abstract】 Objective:To investigate the clinical features and treatment of primary plasma cell leukemia (PPCL).Methods: The data of 22 cases of PPCL patients that were admitted to the first hospital of China Medical University between 1987 and 2009 were retrospectively analyzed.Results:Of the 22 cases,there were 13 males and 9 females.Patient age ranged from 33 to 76 with a mean age of 53.3±13.3 years.The major clinical features were as follows:19 cases had pale appearance,17 cases had fatigue,15 cases had ostealgia,9 cases had fever,9 cases had bleeding,7 cases had weight loss,7 cases had hepatomegaly,11 cases had splenomegaly,7 cases had lymph node enlargement,6 cases had pleural effusion,4 cases had ascites,2 cases had pericardial effusion,2 cases had pelvic effusion,and 3 cases had bilateral edema in the lower extremities.Laboratory examinations showed that WBC increase (68.2%),anemia (86.4%),thrombocytopenia (86.4%),serum M-protein increase,renal insufficiency,hypoproteinemia,and ESR and serum uric acid increase were common.Bone destruction was common.Clinical remission was difficult to achieve or maintain by classic chemotherapy. Ten patients received classic chemotherapy that resulted in temporary complete remission (CR) in 1 patient,partial remission (PR) in 6 patients,and progression in 3 patients.One patient received classic chemotherapy combined with thalidomide and achieved CR for 7 months;3 patients were given Bortezomib combined with chemotherapy or thalidomide,and all of them achieved PR;2 patients were not given chemotherapy due to lung infection,dysfunction of the liver or cardiac insufficiency. One survived for 1 month,while the other survived for 4 months;1 patient died before the therapy started;5 patients abandoned treatment.Conclusion:PPCL has the clinical characteristics of both acute leukemia and multiple myeloma, and is more prone to be acute leukemia with a very poor prognosis.Bortezomib plays an important part in the targeted therapy for PPCL,which provides new hope for PPCL patients.Bortezomib combined with chemotherapy and thalidomide may improve the therapeutic effect.更多还原