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为了洞察特发性难治性铁粒幼红细胞贫血(IRSA)的自然病史,作者详细分析了近18年来收治的37例IRSA病人(含5例RAEB),这些病人年龄28-80岁(平均64岁),骨髓中15%以上有核红细胞是环形铁粒幼红细胞,主要表现红细胞系统异常,但有1/3病人也累及别的细胞系,95%病人骨髓增生活跃伴无效红细胞生成;3例有明显粒细胞生成障碍;2例有明显血小板生成障碍。37例病人的平均生存期为72个月(8-170个月),20%病人在24个月内死亡。少数病人对维生素B6或雄激素治疗有应答,但都是短暂的。15例死者中7例死于心衰(都有继发性血色病);5例转为ANLL后死亡;其余3人分别死于败血症、脑溢血及肝硬化。5例RAEB及2例有第7号染色体单体的病人都渐变为ANLL;3例有第8号染色体三体的
In order to gain insight into the natural history of idiopathic refractory IRB, we analyzed in detail 37 IRSA patients (including 5 RAEB) treated in the past 18 years ranging in age from 28 to 80 years (mean 64 Years old), more than 15% nucleated erythrocytes in bone marrow were ring-shaped erythrocyte erythrocytes, which mainly showed abnormalities of erythrocyte system. However, one third of patients also involved other cell lines, and 95% of patients had active bone marrow hyperplasia with ineffective erythropoiesis. Obvious granulocytogenetic disorders; 2 cases had obvious thrombocytopoiesis. The average survival time of 37 patients was 72 months (8-170 months), and 20% of patients died within 24 months. Few patients respond to vitamin B6 or androgen therapy, but are transient. Seven of the 15 deceased patients died of heart failure (all had secondary hemochromatosis); 5 died of ANLL; and the remaining 3 died of sepsis, cerebral hemorrhage and cirrhosis. 5 cases of RAEB and 2 cases of chromosome 7 patients with a gradual change to ANLL; 3 cases with chromosome 8 trisomy