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遗传性球形红细胞增多症(hereditary spherocytosis,HS)是因先天性遗传性红细胞膜缺陷引起的一种慢性溶血性疾病,多为常染色体显性遗传。主要表现为贫血、黄疸、脾脏增大、外周血球形红细胞显著增多、红细胞渗透脆性增加、慢性贫血过程伴急性溶血发作。在北欧发病率为1/2 000[1],国内发病率尚无明确报道。目前发病机制尚不完全清楚,现将近年来对HS发病机制的研究及治疗进展综述如下。
Hereditary spherocytosis (hereditary spherocytosis, HS) is a chronic hemolytic disease caused by congenital hereditary erythrocyte membrane defects, mostly autosomal dominant inheritance. The main manifestations of anemia, jaundice, spleen increased, peripheral blood cells significantly increased red blood cells, increased penetration of red blood cell fragility, chronic anemia with acute hemolytic attack. In Northern Europe, the incidence is 1/2 000 [1]. The incidence in the country is not yet clearly reported. The pathogenesis is not yet fully understood, and now in recent years the pathogenesis of HS research and progress of treatment are summarized below.