肾静脉梗死，阵发性夜间血红蛋白尿的一种并发症

[Renal vein infarction, a complication of paroxysmal nocturnal hemoglobinuria].

作者信息

de Charry Charlotte, de Charry Félicité, Lemoigne François, Lamboley Jean-Laurent, Pasquet Florian, Pavic Michel

机构信息

Service d'imagerie médicale, hôpital d'instruction des armées Desgenettes, 108, boulevard Pinel, 69003 Lyon, France.

出版信息

Nephrol Ther. 2012 Dec;8(7):537-9. doi: 10.1016/j.nephro.2012.04.001. Epub 2012 May 18.

DOI:10.1016/j.nephro.2012.04.001

PMID:22609136

Abstract

Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) is a rare acquired clonal disorder of the hematopoietic cell, to a somatic mutation in the phosphatidylinositol glycan (PIG-A). The most frequent clinical manifestations are hemolytic crisis and venous thrombosis of the mesenteric, hepatic, portal or cerebral territories. We report a case of paroxysmal nocturnal hemoglobinuria with renal vein thrombosis, a rare complication of this disease.

摘要

阵发性睡眠性血红蛋白尿症（马尔恰法瓦-米凯利病）是一种罕见的获得性造血细胞克隆性疾病，由磷脂酰肌醇聚糖（PIG-A）的体细胞突变引起。最常见的临床表现是溶血危象以及肠系膜、肝、门静脉或脑区的静脉血栓形成。我们报告一例阵发性睡眠性血红蛋白尿症合并肾静脉血栓形成的病例，这是该疾病的一种罕见并发症。