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摘要: Castleman病和POEMS综合征属于罕见病, 二者关系密切, 但临床对其肾脏损伤认识不多。本例患者为44岁男性, 病初表现为顽固性腹腔积液, 之后出现肾功能异常及下肢水肿, 查体发现颈胸部多发血管瘤, 炎症指标及血清血管内皮生长因子明显升高, M蛋白阴性。皮肤活检示肾小球样血管瘤, 肾活检表现为内皮细胞病及小管间质损害, 淋巴结活检诊断为Castleman病。加用足量泼尼松和环磷酰胺后, 病情迅速缓解, 肾功能恢复正常。
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关键词:
- Castleman病 /
- POEMS综合征 /
- 急性肾损伤 /
- 腹腔积液 /
- 肾小球样血管瘤
Abstract: Castleman disease and POEMS syndrome are both rare diseases, and there is a close relationship between each other. A#60hough these two diseases have been investigated for decades, a comprehensive understanding of renal manifestations is still lacking. We reported a 44-year-old man who suffered from refractory ascites initially, and then developed renal insufficiency and lower limb edema. Physical examination at admission indicated mu#60iple hemangioma in the zone of neck and chest. There was a significant elevation in inflammatory indices and serum vascular endothelial growth factor, while the monoclonal protein was negative. The lymph node biopsy showed Castleman disease; and the skin biopsy showed glomeruloid hemangioma. Renal biopsy demonstrated endotheliopathy and tubular-interstitial injury. He was diagnosed with Castleman disease; and after the treatment with high dose glucocorticoid and immunosuppressants, the disease had been relieved rapidly and the renal function returned normal.-
Key words:
- Castleman disease /
- POEMS syndrome /
- acute kidney injury /
- ascites /
- glomeruloid hemangioma
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表 1 POEMS综合征梅奥诊断标准(2007年)
标准 内容 强制性主要标准 多发性神经病(典型为脱髓鞘病变) 单克隆浆细胞增殖性疾病(几乎皆为λ) 其他主要标准 Castleman病 硬化性骨损害 血清/血浆血管内皮生长因子升高 次要标准 器官肿大(脾肿大、肝肿大或淋巴结肿大) 内分泌异常(肾上腺、甲状腺、垂体、性腺、甲状旁腺、胰腺) 血管外容量超负荷(水肿、胸腔积液或腹腔积液) 皮肤改变(色素沉着、多毛、肾小球样毛细血管瘤、多血症、紫绀、潮红、指甲发白) 视乳头水肿 红细胞增多, 血小板增多 其他症状及体征 杵状指, 体重下降, 多汗, 肺动脉高压/限制性肺部疾病, 血栓倾向, 腹泻, VB12水平降低 注：明确诊断需满足2条强制性主要标准，1条其他主要标准，1条次要标准 
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