Rubins J, Young L E
JAMA. 1977 Feb 21;237(8):797-8.
The first reported case of hereditary spherocytosis (HS) and glucose-6-phosphate dehydrogenase deficiency in a black is presented. The recent literature is reviewed, with emphasis on the frequency of multiple inherited RBC defects in this ethnic group. Despite a coexisting hemoglobinopathy or enzyme deficiency, HS can be diagnosed in most cases by the peripheral blood smear, osmotic fragility curve, and family history. The implications of the double RBC abnormality are discussed, stressing the importance of splenectomy in relieving the hemolytic component due to spherocytosis.
本文报告了首例黑人遗传性球形红细胞增多症（HS）和葡萄糖-6-磷酸脱氢酶缺乏症患者。对近期文献进行了综述，重点关注该种族群体中多种遗传性红细胞缺陷的发生率。尽管存在并存的血红蛋白病或酶缺乏症，但大多数情况下，HS可通过外周血涂片、渗透脆性曲线和家族史进行诊断。文中讨论了双重红细胞异常的影响，强调了脾切除术在缓解球形红细胞增多症所致溶血成分方面的重要性。
