摘要
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。
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.
出处
《实用诊断与治疗杂志》
2005年第7期488-489,491,共3页
Journal of Practical Diagnosis and Therapy
