Sharma Sunita, Toppo Anupa, Rath B, Harbhajanka Aparna, Lalita Jyotsna P
Indian J Hematol Blood Transfus. 2010 Sep;26(3):101-2. doi: 10.1007/s12288-010-0034-2. Epub 2010 Oct 17.
Wilson's disease is a rare inherited disorder of copper metabolism causing severe damage to vital organs. Liver and brain disorders are the main manifestations. Severe hemolytic anemia is an unusual complication of Wilson's disease. We present a case who developed spherocytic acute hemolytic anemia (Coomb's negative) as the initial manifestation of Wilson's disease. On examination Kayser- Fleischer ring was found. Laboratory data supported a diagnosis of Wilson's disease.
威尔逊氏病是一种罕见的铜代谢遗传性疾病,会对重要器官造成严重损害。肝脏和脑部疾病是主要表现。严重溶血性贫血是威尔逊氏病的一种罕见并发症。我们报告一例以球形红细胞急性溶血性贫血(库姆斯试验阴性)为威尔逊氏病首发表现的病例。检查时发现了凯-弗环。实验室数据支持威尔逊氏病的诊断。