POEMS综合征的临床与周围神经病理分析

目的：探讨POEMS综合征的临床和周围神经病理特点。方法：回顾性分析5例POEMS综合征的临床特征和4例周围神经活检结果。结果：5例患者临床上均有感觉运动性周围神经损害和肢端凹陷性水肿，腹水3例，胸腔积液2例，心包积液2例。肝脏肿大2例，脾脏肿大3例，淋巴结肿大2例。皮肤变黑5例，多毛症3例。4例腓肠神经活检中，3例神经束膜增厚，束间小血管增多，周围散在有单核细胞。4例均可见脱髓鞘改变和有髓纤维减少，3例可见到少数纤维轴索变性。单神经纤维剥离检查4例，均可见阶段性脱髓鞘改变和薄髓纤维。结论：POEMS综合征是一组以多发性神经病、脏器肿大、内分泌病变、M蛋白、皮肤损害等多系统损害的临床症候群。周围神经病理改变主要为薄髓纤维和脱髓鞘改变，晚期可见轴索变性。

Clinical and pathological study on patients with POEMS syndrome

Objective To explore the clinical and pathological features in POEMS syndrome. Methods The clinical data and the results of biopsy in patients with POEMS syndrome were retrospec- tively studied. Results Among the 5 cases, there were 5 cases with peripheral sensorimotor polyneu- ropathy and 3 cases with ascites, 2 cases with pleural effusions, 2 cases with hepatomegaly, 3 cases of splenomegaly, one case with lymphadenopathy and 3 cases with hypertrichosis.In the 4 sural nerve biopsy specimens, there were 3 cases with a mixture of both axonal degeneration and segmental de- myelination, segmental demyelination alone in one case. Conclusion POEMS syndrome is a group of clinical symptom characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin hyperpigmentation. Peripheral nerve pathology includes segmental demyelination and axonal de- generation.