自体外周血造血干细胞移植治疗POEMS综合征

Autologous peripheral blood stem cell transplantation for POEMS syndrome

回顾性分析采用自体外周血造血干细胞移植(auto-PBSCT)治疗POEMS综合征的疗效及安全性。6例患者用大剂量马法兰预处理后进行auto-PBSCT治疗,干细胞动员采用环磷酰胺后注射粒细胞集落刺激因子,其中1例以胸腹水为首发表现的患者动员前予Rd方案(来那度胺+地塞米松)治疗。患者耐受性好,移植后均迅速造血重建,无移植相关死亡,3个月后血清血管内皮生长因子水平明显下降/恢复正常,神经系统缓解的比例为5/6。

Six patients with POEMS syndrome who received autologous peripheral blood stem cell transplantation (auto-PBSCT) were retrospectively analyzed. Conditioning regimen was high dose melphalan. Peripheral blood stem cells were collected after mobilization with cyclophosphamide (CTX) and growth factors. One patient presenting hydrothorax and ascites was treated with 3 cycles of lenalidomide and dexamethasone before mobilization. Auto-PBSCT was fairly tolerable. Hematopoietic reconstitution was successful in all patients without transplantation-related mortality. A decrease or normalization of serum vascular epithelial growth factor (VEGF) was observed in all patients at 3 months after transplantation. The neurological remission was seen in 5/6 patients.