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遗传性球形红细胞增多症(hereditaryspherocytosis,HS),是一种先天性红细胞膜骨架蛋白异常引起的遗传性溶血性疾病。其主要特点是外周血中见到较多小球形红细胞。临床上以贫血、黄疸、脾肿大、血液中球形红细胞增多、病程呈慢性贫血经过并伴有溶血反复急性发作为主要特征。大多数患者家族中有同样病例,多为常染色体显性遗传,少数为常染色体隐性遗传。现就同一家族病患的治疗体会进行总结如下。
Hereditarium hypersensitivity (hereditaryspherocytosis, HS), is a hereditary hemolytic disease caused by abnormality of congenital erythrocyte membrane scaffold protein. Its main feature is seen in peripheral blood more small red blood cells. Clinical anemia, jaundice, splenomegaly, blood spherocytosis, the course of chronic anemia was accompanied by recurrent hemolytic acute episodes as the main feature. Most patients have the same family of cases, mostly autosomal dominant, a few autosomal recessive. The treatment of the same family members are summarized below.