POEMS综合征的临床和周围神经病理特点

  目的  总结POEMS综合征的临床及周围神经活检病理特点。  方法  回顾性分析北京协和医院11例行腓肠神经活检的POEMS综合征患者的临床表现及腓肠神经活检病理特点。  结果  11例患者中, 男8例, 女3例, 年龄40~64岁, 病程8个月~8年。11例患者均表现为感觉运动性周围神经病, 10例可见单克隆免疫球蛋白, 其中IgAλ 6例, IgGλ 2例, 未分型λ轻链2例。10例有脏器肿大, 8例有内分泌改变, 10例有皮肤损害。腓肠神经活检结果均有异常发现。其中3例神经纤维轻度减少, 8例神经纤维中重度减少; 7例以大纤维病变为重, 2例以小纤维病变为主, 2例大小纤维受累程度相当。光镜下, 5例以轴索变性为主, 6例表现为混合性轴索髓鞘性周围神经病, 可见薄髓纤维或髓鞘厚度不均。3例可见再生神经丛。11例均可见神经束间或神经内膜血管增多, 4例可见血管基底膜增厚, 未见炎细胞浸润。4例可见神经束膜下间隙增宽, 2例可见神经内膜水肿。  结论  POEMS综合征患者周围神经病变表现为对称性远端感觉运动性周围神经病; 神经病理改变为轴索变性或混合性轴索髓鞘病变, 伴有小血管病变, 无明确炎性改变。

Retrospective Analysis of Clinical and Pathological Characteristics of POEMS Syndrome in 11 Patients

Objective To summarize the clinical features and neuropathological characteristics of peripheral nerves in patients with POEMS syndrome.Methods We retrospectively analyzed the clinical and neuropathological characteristics of 11 patients with POEMS syndrome who underwent electrophysiology and sural nerve biopsy in our department from January 2003 to December 2008.Results Among these 11 patients,there were 8 men and 3 women,aged 40 to 64 years,with a disease course ranging from 8 months to 8 years.All patients suffered from sensorimotor polyneuropathy.Monoclonal immunoglobulins were detected in 10 patients,including IgAλ(n=6),IgGλ(n=2),and monoclonal λ light chain with unknown typing(n=2).Ten patients had organomegaly,8 had endocrine disease,and 10 had skin changes.Biopsy of sural nerve presented loss of myelinated fibers.Five showed axonal degenation and six showed both axonal degeneration and demyelination.Changes in the endoneurial and epineurial microvessels,including hyperplasia of endothelial cells and widened basement membrane,were observed.Conclusion Biopsy of the sural nerve in patients with POEMS syndrome shows both axonal degeneration and demyelination or axonal degeneration,as well as small vessel lesions without inflammation.