POEMS 综合征24例临床分析

 【摘要】　目的　分析POEMS综合征的临床特征，提高对其诊断、治疗的认识，探索有效的治疗策略。方法　回顾性分析1997年9月至2011年1月24例POEMS综合征患者的临床特点、实验室检查及治疗方法，并复习相关文献。结果　24例中男18例，女6例;年龄40～73岁，中位年龄56.5岁。24例患者均有周围神经病变，19例患者出现肝大，17例患者出现脾大，15例患者出现内分泌改变，18例患者血清M蛋白阳性，21例患者出现皮肤色素沉着。治疗以MP方案联合沙利度胺为主，有效率达100 %。1例行外周血自体造血干细胞移植（PBSCT）2年，周围神经系统症状达到完全缓解，M蛋白持续阴性。结论　POEMS综合征为少见的多系统损害疾病，对于以周围神经病变为主要首发症状的病因不明患者，应进一步筛查，提高临床检出率。左旋苯丙氨酸氮芥联合泼尼松为最有效的治疗方案，PBSCT为POEMS综合征的治疗提供了新的研究方向。

Clinical analysis of 24 patients with POEMS syndrome

Objective To analyze the characters of POEMS syndrome, raise physician awareness of diagnosis and therapy, and explore the optimal treatment strategies. Methods The clinical features, laboratory examination and therapy of 24 patients with POEMS syndrome were analyzed, and relative literatures were reviewed. Results A strong predominance of male over female was found, 18 vs 6. All patients were over 40 years old, with a mean age 56.5 years old, indicating a common adult involvement. All presented with polyneuropathy, which was also the most common complaints when admitted, which reminded neurologists of underlying possibility of a rare plasma cell disorder. Organomegaly was found, including 19 cases with hepatomegaly, 17 patients with splenomegaly. Endocrinal abnormalities were also found in 15 cases. 18 patients were M protein positive. Skin pigmentation was recognized in 21 cases. Melphalan in combination with prednisone was applied and 100 % response was observed. One of the patients received peripheral blood stem cell transplantation （PBSCT） 2 years ago and a durable response was observed with a continuous complete remission of polyneuropatby and the absence of M protein following 2 year post PBSCT. Conclusion POEMS syndrome is a rare multisystem disorder, the combination of symptoms and signs is highly complex, which is easy to misdiagnose or missed diagnose. A polyneuropathy with either organomegaly, endocrinal abnormalities, skin disorder or serositis is required a further investigation of clonal M protein and bone marrow. Melphalan and prednisone probable are the optimal regimens. PBSCT provides a new choice for therapy and research of POEMS syndrome.