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Ⅱ型冷球蛋白血症导致具有肾脏意义的单克隆丙种球蛋白病。

Type II Cryoglobulinaemia causing Monoclonal Gammopathy of Renal Significance.

机构信息

Department of Renal Medicine, Basildon and Thurrock University Hospitals NHS Foundation Trust.

Department of Cellular Pathology, Royal London Hospital, Barts Health NHS Trust.

出版信息

Acute Med. 2021;20(3):227-230.

Abstract

A 53 year old female with a background of hypertension, hypothyroidism and Raynaud's was admitted with an acute ischaemic stroke and referred to the renal team after a routine urine dip revealed microscopic haematuria and nephrotic-range proteinuria. Blood tests revealed renal impairment, a monoclonal IgM kappa paraprotein, low complement C4 concentration and a positive rheumatoid factor. Active cryoglobulinaemia was suspected and testing demonstrated type II cryoglobulins secondary to the monoclonal IgM kappa paraprotein. Bone marrow biopsy was normal. Renal biopsy revealed cryoglobulinaemia associated membranoproliferative glomerulonephritis. Treatment with steroids and rituximab improved renal function and proteinuria. This case fits within the evolving spectrum of disorders now termed Monoclonal Gammopathy of Renal Significance and highlights the value of biopsying and treating these patients early.

摘要

一位 53 岁女性,有高血压、甲状腺功能减退和雷诺现象病史,因急性缺血性中风入院,常规尿液检查发现镜下血尿和肾病范围蛋白尿后转至肾脏科。血液检查显示肾功能损害、单克隆 IgM kappa 副蛋白、补体 C4 浓度低和类风湿因子阳性。怀疑存在活动性冷球蛋白血症,检测显示继发于单克隆 IgM kappa 副蛋白的 II 型冷球蛋白血症。骨髓活检正常。肾活检显示冷球蛋白血症相关的膜增生性肾小球肾炎。类固醇和利妥昔单抗治疗改善了肾功能和蛋白尿。该病例符合目前称为肾脏意义单克隆丙种球蛋白病的不断演变的疾病谱,并强调了早期对这些患者进行活检和治疗的价值。

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