遗传性球形红细胞增多症与α地中海贫血的相互作用：一项家族研究。
Interaction of hereditary spherocytosis and alpha thalassaemia: a family study.
作者信息
Li C K, Ng M H, Cheung K L, Lam T K, Shing M M
机构信息
Department of Paediatrics, Prince of Wales Hospital, Chinese University of Hong Kong.
出版信息
Acta Haematol. 1994;91(4):201-5. doi: 10.1159/000204335.
We report here a family of patients with coexistent hereditary spherocytosis and alpha-thalassaemia. The different clinical presentations of the family members were affected by the severity of the alpha-thalassaemia. The haemolytic effect of hereditary spherocytosis was modulated by the increased osmotic resistance of thalassaemia in the patients with both disorders. Coexistence of haemoglobin H disease and hereditary spherocytosis resulted in an asymptomatic state. In contrast, coexistence of alpha-thalassaemia trait and hereditary spherocytosis was still haemolytic. The different shapes of the osmotic curves in the family members correlated with the severity of their symptoms.
我们在此报告一个患有遗传性球形红细胞增多症和α地中海贫血并存的患者家族。家族成员的不同临床表现受α地中海贫血严重程度的影响。在同时患有这两种疾病的患者中，地中海贫血增加的渗透压抵抗力调节了遗传性球形红细胞增多症的溶血作用。血红蛋白H病和遗传性球形红细胞增多症并存导致无症状状态。相比之下，α地中海贫血特征与遗传性球形红细胞增多症并存仍会发生溶血。家族成员中不同形状的渗透曲线与其症状的严重程度相关。
Zotero 插件