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Retrospective Analysis of Clinical and Pathological Characteristics of POEMS Syndrome in 11 Patients
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摘要:
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。 Abstract: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 ves-sel lesions without inflammation. -
Key words:
- POEMS syndrome /
- sural nerve /
- biopsy /
- monoclonal immunoglobulin
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图 1 POEMS综合征患者腓肠神经活检可见轴索变性及薄髓纤维
A.病例3光镜下可见较多亚急性轴索变性，部分髓球形成(髓鞘染色，×400); B.病例3电镜下可见轴索变性(×1500); C.病例11电镜下可见薄髓纤维(×1500)
图 2 POEMS综合征患者神经活检可见神经外膜血管增多，血管壁增厚
A.病例10光镜下可见神经束间血管明显增多(HE，×40); B.病例6光镜下可见神经束间血管管壁增厚(HE，×100); C.病例6电镜下可见血管内皮细胞增生，基底膜增厚(×3000)
表 1 11例POMES综合征患者临床特点及实验室检查结果
病例 性别 年龄(岁) 总病程/周围
神经病病程(月)脏器
肿大内分泌
改变皮肤
病变M蛋白
类型血小板
(× 109 /L)腰穿脑脊液 压力(mm H20) 蛋白(g/L) 1 男 57 96/60 + + + IgAλ NV 245 1.15 2 男 52 36/36 + + + IgAλ 678 265 1.76 3 男 43 11/11 + - + IgGλ 362 NE 1.42 4 女 42 36/24 + - + IgAλ 399 NE NE 5 男 42 12/12 - + - λ NV NV 1.63 6 男 58 48/36 + + + IgGλ NV NV 1.63 7 男 64 8/ 8 + + + IgAλ 464 240 1.46 8 女 52 10/ 3 + + + λ 510 NE NE 9 男 50 72/ 5 + + + - 463 NV 1.16 10 女 40 36/36 + + + IgAλ NV 300 0.76 11 男 64 20/20 + - + IgAλ 520 NE NE + :阳性表现; － :阴性表现; NV:正常范围; NE:未检测; 1 mm H20 = 0. 0098 kPa; 脑脊液蛋白正常值: 0. 15 ~ 0. 45 g/L 
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表 2 11例POEMS综合征患者腓肠(腓浅)神经活检结果
病例 病变时相 病变程度 病变类型 受累神经纤维类型 1 慢性 重度 轴索变性和脱髓鞘 大纤维为主 2 慢性 中度 轴索变性和脱髓鞘 大纤维为主 3 亚急性 中度 轴索变性 大小纤维均受累 4 慢性 中度 轴索变性 大纤维为主 5 亚急性 重度 轴索变性和脱髓鞘 小纤维为主 6 慢性 中度 轴索变性 大纤维为主 7 慢性 中度 轴索变性 大纤维为主 8 亚急性 重度 轴索变性和脱髓鞘 大小纤维均受累 9 慢性 轻度 轴索变性 大纤维为主 10 慢性 轻度 轴索变性和脱髓鞘 大纤维为主 11 慢性 轻度 轴索变性和脱髓鞘 小纤维为主
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