Sickle cell disease is a hereditary chronic hemolytic anaemia mostly found in negroes. The blood of these patients contains hemoglobin S. Absence of oxygen determines the sickling to the red blood cells. In the heterozygous state clinical manifestations usually are absent. The homozygous form shows different symptoms. The progressive course with hemolytic, aplastic or thrombotic crises mostly ends in an early death in infancy or childhood. Treatment is largely palliative. Transfusions, treatment of the crises and general measures play the most essential role.
镰状细胞病是一种遗传性慢性溶血性贫血,多见于黑人。这些患者的血液中含有血红蛋白S。缺氧会导致红细胞镰变。杂合子状态通常无临床表现。纯合子形式则表现出不同症状。其病程呈进行性,伴有溶血性、再生障碍性或血栓形成性危象,大多在婴儿期或儿童期早期死亡。治疗主要是姑息性的。输血、危象治疗和一般措施起着最重要的作用。