POEMS综合征患者的临床特征及疗效分析

The Clinical Characteristics and Outcomes of the Patients with POEMS Syndrome

目的:分析POEMS综合征患者的临床特征,并探讨有效的治疗策略。方法:对2012年6月至2018年6月本院收治的75例POEMS综合征患者的病历资料进行收集并回顾性分析,总结患者的临床特点、治疗方法及疗效。结果:75例POEMS患者诊断时中位年龄50(30-81)岁,100%(75/75)见周围神经病变,77.3%(58/75)合并器官肿大,82.7%(62/75)伴有内分泌功能异常,93.3%(70/75)存在单克隆性浆细胞疾病,64.0%(48/75)伴有皮肤改变。75例患者中,5例放弃治疗,剩余70例患者经治疗后症状得到不同程度的改善,血液学完全缓解(CR_(H))率为28.6%,神经系统缓解(R_(N))率为85.7%。BD、RD方案在CR_(H)及R_(N)方面疗效优于VAD、COP方案(P<0.05)。其中2例患者于BD方案化疗后序贯自体造血干细胞移植,移植后周围神经症状改善显著,M蛋白转阴,疗效理想。70例患者随访的中位时间为42(4-103)个月,2年总生存率(OS)为86.7%,5年OS为81.0%。结论:POEMS综合征临床表现复杂多样,临床医师应提高警惕,减少误诊、漏诊。硼替佐米或来那度胺等药物可用于一线治疗,有条件者建议行自体造血干细胞移植。

Objective:To analyze the clinical characteristics of patients with POEMS syndrome and explore its effective treatment strategies.Methods:The clinical data of 75 patients with POEMS syndrome treated in The First Affiliated Hospital of Zhengzhou University from June 2012 to June 2018 were collected and retrospectively analyzed.The clinical characteristics,treatment regimes and outcomes of the patients were summarized.Results:The median age of 75 diagnosed patients was 50(30-81)years old and 100%(75/75)of the patients were accompanied with peripheral neuropathy,77.3%(58/75)with organ enlargement,82.7%(62/75)with endocrine abnormality,93.3%(70/75)with monoclonal plasma cell diseases and 64.0%(48/75)with skin changes.Among the 75 patients,5 cases gave up treatment,while the others showed varying degrees of improvement after treatment.The hematological complete remission(CR_(H))rate of the 70 patients was 28.6%and the rate of neurological remission(R_(N))was 85.7%.BD and RD regimens showed better efficacy in CR_(H)and R_(N)than old schemes,such as VAD and COP(P<0.05).Two patients underwent sequential autologous hematopoietic stem cell transplantation(HSCT)after BD chemotherapy and achieved ideal therapeutic effects,including the significant improvement of peripheral neurological symptoms and the M protein turned negative.The median follow-up time of the 70 patients was 42(4-103)months,while the 2-year overall survival rate(OS)was 86.7%,and the 5-year OS was 81.0%.Conclusion:The clinical manifestations of POEMS syndrome are complex and diverse,the clinicians therefore should be vigilant to reduce the misdiagnosis and missed diagnosis.Bortezomib or Lenalidomide can be recommended as the first-line medicines and autologous HSCT should be considered for appropriate patients.