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具有肾脏意义的单克隆丙种球蛋白病伴发Ⅰ型冷球蛋白血症相关的严重血栓性微血管病。

Monoclonal gammopathy of renal significance presenting with cryoglobulinaemia type I associated severe thrombotic microangiopathy.

作者信息

Hesius Eva, Bunthof Kim, Steenbergen Eric, de Kort Elizabeth, Klein Inge, Wetzels Jack

机构信息

Radboudumc, Department of Haematology, Nijmegen, The Netherlands.

Radboudumc, Department of Nephrology, Nijmegen, The Netherlands.

出版信息

Clin Kidney J. 2022 Mar 12;15(7):1425-1428. doi: 10.1093/ckj/sfac078. eCollection 2022 Jul.

Abstract

We report a 53-year-old man who presented with acute renal failure. His medical history revealed a spondyloarthropathy, for which secukinumab was started recently, and a monoclonal gammopathy of unknown significance. Kidney function deteriorated despite the withdrawal of secukinumab and dialysis was started. In the serum, type 1 cryoglobulins were present and a kidney biopsy showed ischaemic glomeruli, with thrombosis of the larger interlobular arteries. Other causes of thrombotic microangiopathy were excluded. Bone marrow immunophenotyping showed 1% monoclonal plasma cells. A diagnosis of monoclonal gammopathy of renal significance was made. Haematological treatment resulted in haematological and renal response.

摘要

我们报告了一名53岁的男性,他出现了急性肾衰竭。他的病史显示患有脊柱关节病,最近开始使用司库奇尤单抗治疗,以及意义未明的单克隆丙种球蛋白病。尽管停用了司库奇尤单抗,肾功能仍恶化,于是开始进行透析。血清中存在1型冷球蛋白,肾脏活检显示肾小球缺血,较大的小叶间动脉有血栓形成。排除了血栓性微血管病的其他病因。骨髓免疫表型分析显示有1%的单克隆浆细胞。诊断为具有肾脏意义的单克隆丙种球蛋白病。血液学治疗导致血液学和肾脏方面的缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e78/9217659/5936f4f1864d/sfac078fig1.jpg

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