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目的 探讨浆细胞白血病 (PCL)的临床特点、疗效及预后。方法 对 8例PCL的临床表现及实验进行回顾性分析 ,观测浆细胞直径及形态与多发性骨髓瘤 (MM )细胞进行比较。结果 8例PCL有如下特点 :(1)起病急骤 ,8例患者确诊前平均病程为 2 7个月。 (2 )发病率男性明显多于女性 (7∶1) ,PPCL多于SPCL(7∶1)。 (3)常有发热、骨痛、出血、体重减轻等表现 ,伴有胸骨压痛、肝脾淋巴结肿大者远比MM发生率高。 (4 )骨质破坏率较MM增高。 (5 )球蛋白多数增高 ,血沉明显增快 ,尿素氮升高或出现蛋白尿 ,此与MM相似。 (6 )血液化验多数患者贫血明显 ,血小板降低 ,而白细胞升高。 (7)PCL细胞多数体积偏小 ,核浆比例大。疗效 :3例未经治疗者 3个月内死亡 ,5例接受治疗者 1例完全缓解 (CR) ,2例部分缓解 (PR) ,2例无效。其中 1例持续CR已 6年 5个月并恢复工作 ,1例PR达 2年 11个月 ,仍在治疗中。结论 PCL是类似于MM的急性白血病 ,确诊后尽早采用以烷化剂为主的联合化疗 ,并坚持系统治疗 ,部分患者可以获得长生存。
Objective To investigate the clinical characteristics, curative effect and prognosis of plasma cell leukemia (PCL). Methods The clinical manifestation and experiment of 8 cases of PCL were retrospectively analyzed. The diameter and morphology of plasma cells were compared with that of multiple myeloma (MM) cells. Results 8 cases of PCL has the following characteristics: (1) sudden onset, 8 patients with an average of 27 months before diagnosis. (2) The incidence was significantly higher in males than in females (7: 1), with more PPCL than SPCL (7: 1). (3) Often fever, bone pain, bleeding, weight loss and other performance, accompanied by sternal tenderness, hepatosplenomegaly enlarged than the high incidence of MM. (4) bone destruction rate higher than MM. (5) most of the elevated globulin, erythrocyte sedimentation significantly faster, elevated urea nitrogen or proteinuria, similar to MM. (6) blood test Most patients with anemia, thrombocytopenia, and leukocytosis. (7) The majority of PCL cells are smaller in size and larger in nuclear cytoplasm. Efficacy: 3 patients died within 3 months without treatment, 1 patient had complete remission (CR), 2 patients had partial response (PR), and 2 patients were ineffective in 5 patients. One of the patients had continuous CR for 6 years and 5 months and resumed work. One patient had achieved PR for 2 years and 11 months and was still under treatment. Conclusions PCL is an acute leukemia similar to MM. As soon as possible after diagnosis, alkylating agent-based combination chemotherapy is used and systemic treatment is continued. Some patients can get long-term survival.