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特发性顽固性铁粒幼红细胞性贫血(Idiopathic Refractory Sideroblastic Anemia;IRSA)是一种呈慢性经过的疾病,对各种治疗反应不佳。Dameshek曾提出IRSA中至少有50%的病例将来会变成红白血病。Kushner等(1971)经过详细的复习文献后提出IRSA转变为白血病的最高发生率为7.4%,而比较可靠的发生率为5%左右。因而有人认为IRSA是一种白血病前期状态。血红蛋白H病是珠蛋白α肽链合成障碍的疾病,多余的β肽链聚合为四聚体而形成一种不稳定的血红蛋白——血红蛋白H。绝大多数
Idiopathic Refractory Sideroblastic Anemia (IRSA) is a chronic disease that does not respond well to various therapies. Dameshek has argued that at least 50% of cases in the IRSA will become erythroleukemia in the future. After a detailed review of the literature, Kushner et al. (1971) suggested that the highest incidence of leukemia in IRSA was 7.4% and the more reliable rate was 5%. Some people think that IRSA is a pre-leukemia state. Hemoglobin H disease is a disorder in which the synthesis of globin alpha peptide chains is impaired, and the extra beta peptide chains polymerize into tetramers to form hemoglobin H, an unstable hemoglobin. Mostly