Kanashima Hiroshi, Yoshida Masahiro, Ogawa Yoshihiko, Nakao Takafumi, Nakano Satoshi, Inoue Takeshi, Yamane Takahisa
Dept. of Hematology, Osaka City General Hospital.
Gan To Kagaku Ryoho. 2013 Apr;40(4):503-6.
The patient, a 44-year-old male, subjectively noticed edema in his lower legs in March, 2010. By June 2010, he could not walk, and was admitted to this hospital. Since multiple neuropathy, increased serum vascular endothelial growth factor-3, M-proteinemia, edema, pericardial effusions, papilledema, increased platelet count and skin lesions were detected in the complete examination, he was diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-proteinemia and skin changes (POEMS) syndrome. Treatment with steroid and furosemide was initiated in September 2010. This markedly improved edema and brought about mild recovery of proximal lower-limb muscle strength. The patient, however, suffered prolonged difficulty in walking because his distal lower-limb muscle strength was not improved. In October 2010, he received treatment with high-dose cyclophosphamide, followed by peripheral blood stem cell sampling with granulocyte-colony stimulating factor. In November 2010, he underwent treatment with high-dose melphalan, followed by autologous peripheral blood stem cell transplantation. Recovery to neutrophils greater than 500 was achieved at 13 days after transplantation. He could walk without assistance in February 2011. M-protein was not identified by immunofixation electrophoresis in August 2011. Autologous peripheral blood stem cell transplantation is regarded as a useful treatment for POEMS syndrome.
患者为一名44岁男性,于2010年3月自觉小腿出现水肿。到2010年6月,他已无法行走,遂入住本院。由于在全面检查中发现有多发性神经病变、血清血管内皮生长因子-3升高、M蛋白血症、水肿、心包积液、视乳头水肿、血小板计数增加及皮肤病变,他被诊断为多发性神经病变、脏器肿大、内分泌病、M蛋白血症及皮肤改变(POEMS)综合征。2010年9月开始使用类固醇和速尿进行治疗。这显著改善了水肿,并使下肢近端肌肉力量稍有恢复。然而,患者仍长期存在行走困难,因为其下肢远端肌肉力量未得到改善。2010年10月,他接受了大剂量环磷酰胺治疗,随后使用粒细胞集落刺激因子进行外周血干细胞采集。2010年11月,他接受了大剂量美法仑治疗,随后进行了自体外周血干细胞移植。移植后13天中性粒细胞恢复至大于500。2011年2月他能够独立行走。2011年8月免疫固定电泳未检测到M蛋白。自体外周血干细胞移植被认为是治疗POEMS综合征的一种有效方法。