摘要
本研究旨在探讨骨硬化性骨髓瘤(POEMS综合征)的临床特征,评价沙利度胺联合VAD方案治疗POEMS综合征的疗效。回顾性分析2006年6月至2011年7月我院收治的27例POEMS综合征患者的临床表现、实验室检查、治疗及预后情况。结果表明:27例POEMS患者具有多发性周围神经病变(27/27)、肝脾淋巴结肿大(15/27)、内分泌改变(24/27)、皮肤病变(22/27)等典型临床表现及血清M蛋白阳性(23/27);此外,还有周围性水肿、浆膜腔积液、视盘水肿、骨硬化病变等。用沙利度胺联合VAD方案治疗POEMS综合征缓解率分别为脏器肿大60%,水肿58.3%,皮肤病变41%,内分泌病变45.8%;治疗后IgG/λ型、IgA/λ型患者血清M蛋白水平均显著下降,与治疗前相比差异有统计学意义(P<0.05);神经系统ODSS值与治疗前相比差异有统计学意义(P<0.01)。结论:POEMS综合征临床特征复杂易误诊,血清M蛋白阴性的疑诊患者应积极寻找单克隆浆细胞增生的证据;用VAD方案联合沙利度胺治疗POEMS综合征疗效明显,副作用少,患者耐受性好,且安全性较高,在临床可作为首选的治疗途径。
This study was purposed to analyze the clinical features and evaluate the efficacy of thalidomide combined with VAD regimen for treatment of osteosclerotic myeloma（POEMS syndrome）.The data of 27 patients with POEMS syndrome in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively,including clinical manifestations,laboratory tests,treatments and prognosis.The results showed that the polyneropathy was observed in 27 patients（27/27）,hepato-spleno-lymphadenectasis was found in 15 patients（15/27）,endocrinopathy was found in 24 patients（24/27）,skin changes was observed in 22 patients（22/27）.M protein was found in 23 patients（23/27）;in addition to these clinical manifestations,the papilledema serous cavity effusion and sclerotic bone lesion were also freguently observed in patients with POEMS syndrome.The remission rates of treatment of POEMS syndrome with thalidomide combined with VAD regimen for organomegaly,edema,skin changes,and endocrinopathy were 60%,58.3%,41% and 45.8% respectively.The level of serum M protein and the nervous system ODSS value decreased greatly after treatment（P0.01）.It is concluded that the clinical characteristics of POEMS syndrome are complicated and easy to be misdiagnosed,and the evidence of monoclonal plasma cell hyperplasia should be actively searched for those patients whose serum M protein is negative.Thalidomide combined with VAD regimen for trentment of patients with POEMS syndrome has advantages such as significant curative effects,less side-effects,good tolerance,and higher safety and can be chosen as a preferred approach.
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2012年第2期348-351,共4页
Journal of Experimental Hematology
作者简介
通讯作者：孙慧，主任医师，教授，博导．电话：（0371）66295093E—mail：sunhui371@medmail．com．cn
