遗传性球形细胞增多症是一种先天性溶血性疾病。其主要特点是:(1)红细胞破坏率增加,(2)球形细胞增多,(3)脾脏肿大,(4)家族性发病,(5)脾切除几乎都有效,过多的红细胞破坏能造成临床上的贫血,球形细胞增多,血清铁浓度升高,溶血性黄疸,骨胳异常,慢性或复发性小腿溃疡和胆石病。作者研究了1960～1979的20年中,在Johns Hopkins医院因此病而行脾切除手术的全部病例,讨论了病理生理,外科处理和关于胆道合併症的处理建 Hereditary spherocytosis is a congenital hemolytic disease. Its main features are: (1) increase in the rate of destruction of red blood cells, (2) increase in spherical cells, (3) enlargement of the spleen, (4) familial onset, (5) splenectomy almost all effective excessive destruction of red blood cells can cause Clinical anemia, increased spherical cells, elevated serum iron levels, hemolytic jaundice, skeletal abnormalities, chronic or recurrent leg ulcers and cholelithiasis. The authors studied all cases of splenectomy for this disease at Johns Hopkins Hospital during the two decades from 1960 to 1979 and discussed pathophysiology, surgical management, and management of biliary complications