[Successful treatment of POEMS syndrome with high-dose chemotherapy and autologous peripheral blood stem cell transplantation].
[Successful treatment of POEMS syndrome with high-dose chemotherapy and autologous peripheral blood stem cell transplantation].
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大剂量化疗联合自体外周血干细胞移植成功治疗POEMS综合征[J].
DOI：
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发表时间：
2004
期刊：
影响因子：
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通讯作者：
Tori Kurasaki
中科院分区：
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作者：
K. Takai;K. Niikuni;Tori Kurasaki
一名 42 岁女性因心包和胸腔积液、腹水和不明原因的主动脉旁淋巴结肿大就诊。六个月后，她因发热、下肢疼痛和运动障碍、积液、腹水和水肿加重而入院。体格检查可见肝脾肿大、皮肤色素沉着和多毛症。免疫电泳显示血清中存在单克隆 IgA-lambda 蛋白，尿液中存在 Bence-Jones 蛋白-lambda。骨髓穿刺显示非典型浆细胞轻度增加。血管内皮生长因子 (VEGF) 显着增加至 10,900 pg/ml。肌电图显示提示脱髓鞘的变化。这些临床特征与 POEMS 综合征的诊断一致。 VAD 化疗对积液和神经性恶化无效。通过胸管引流控制大量胸腔积液后，用环磷酰胺和G-CSF进行外周血干细胞（PBSC）采集。患者连续2天接受马法兰100mg/m2，2天后输注PBSC。骨髓迅速恢复，心包、胸腔积液完全消失。她的表现状态从卧床不起的状态明显改善。应进一步研究高剂量美法仑联合自体 PBSCT 作为 POEMS 综合征的推荐疗法。
A 42-year-old woman presented with pericardial and pleural effusion, ascites and para-aortic lymphadenopathy of unknown etiology. Six months later she was admitted with fever, pain and motor disturbance of lower limbs, and exacerbation of the effusion, ascites and edema. Physical examination showed hepatosplenomegaly, skin pigmentation and hypertrichosis. Immunoelectophoresis revealed monoclonal IgA-lambda protein in the serum and Bence-Jones protein-lambda in the urine. Bone marrow aspiration showed a mild increase of atypical plasma cells. Vascular endothelial growth factor (VEGF) had markedly increased to 10,900 pg/ml. Electromyography showed changes suggestive of demyelination. These clinical features were consistent with the diagnosis of POEMS syndrome. VAD chemotherapy was not effective for the effusion and neuropathic deterioration. After control of the massive pleural effusion by chest tube drainage, peripheral blood stem cell (PBSC) collection was performed with cyclophosphamide and G-CSF. The patient received melphalan 100 mg/m2 on 2 consecutive days and the PBSC were infused 2 days later. The bone marrow recovered rapidly and the pericardial and pleural effusion disappeared completely. Her performance status markedly improved from a bedridden state. High-dose melphalan with auto-PBSCT should be investigated further as a recommended therapy for POEMS syndrome.