论文部分内容阅读
自1956年发现葡萄糖6-磷酸脱氢酶缺陷症伴有先天性非球形红细胞溶血性贫血(NSHA)以来,以后陆续发现了红细胞内其他特殊酶缺陷的病例。目前已知有20多种红细胞酶缺陷,其中至少有18种出现急、慢性溶血。本症群的特点:①原则上属于非球形红细胞,②红细胞脆性正常,③血红蛋白无异常,④脾切除可以改善贫血症状,但仍有持续性溶血,⑤属于遗传性疾病。由于近年来生化和检验技术的进展,对这些症群的研究结果,大既上可分为两大类:
Since the discovery of glucose 6-phosphate dehydrogenase deficiency accompanied by congenital non-spherical hemolytic anemia (NSHA) in 1956, one after another found cases of other special enzyme defects in erythrocytes. There are currently more than 20 kinds of red blood cell enzyme defects, of which at least 18 kinds of acute and chronic hemolysis. The characteristics of this disease group: ① in principle belong to non-spherical red blood cells, ② erythrocyte fragility normal, ③ hemoglobin no abnormalities, ④ splenectomy can improve anemia symptoms, but there are still persistent hemolysis, ⑤ belong to genetic disease. Due to the progress of biochemistry and testing technology in recent years, the research results on these syndromes can be divided into two major categories: