腹腔积液1月余, 肾功能异常2周

Ascites for more than One Month, Renal Insufficiency for Two Weeks

  • 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。

     

    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.

     

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