补肾益髓法治疗地中海贫血197例生存情况调查

目的调查经补肾益髓法治疗的地中海贫血患者的基本生存情况。方法对197例治疗病例通过电话随访进行患者生存情况调查。结果197例患者总存活率为87．3％，其中重型患者存活率65．71％，中间型患者存活率99．2％，α-地中海贫血患者存活率100％，β-地中海贫血患者存活率85．0％。进行了基因型分析的158例患者中，纯合子患者存活率为63．16％，双重杂合子患者存活率为89．16％，杂合子患者存活率为100％。对于β-地中海贫血患者，存活率重型〈中间型，纯合子〈双重杂合子〈杂合子；对于重型地中海贫血患者，存活率纯合子〈双重杂合子：对于纯合子患者和双重杂合子患者，存活率均重型〈中间型。结论地中海贫血患者的存活率与其地中海贫血类型、临床分型、基因类型有一定的关系，其中受临床分型和基因类型影响最大。

Investigation of Survival Situation of 197 Thalassemic Cases Treated with Bushen Yisui Method

Objective To investigate the survival situation of thalassemia cases treated with Bushen Yisui Method. Methods The survival situation of 197 cases were investigated by telephone. Results The total survival rate of 197 cases is 87.3%, among which the thalassemia major is 65.71%, the thalassemia intermedia is 99.2%, the α-thalassemia is 100%, the β-thalassemia is 85.0%. 158 of 197 cases have been analyzed genotype, among which 63.16% is the survival rate of homozygote, 89.16% of the heterogenic duplex heterozygote, 100% of the heterozygote. To β-thalassemia, the survival rate has these trends： the major 〈 the intermedia, the homozygote 〈 the heterogenic duplex heterozygote 〈 the heterozygote. To thalassemia major, the survival rate has the trend： the homozygote 〈 the heterogenic duplex heterozygote. To homozygote and heterogenic duplex heterozygote, the survival rate has the trend： the major 〈 the intermedia. Conclusion The survival rate of 197 cases has relations with the type of thalassemia, the clinical phenotype and the genotype, and the clinical phenotype and the genotype have the most effects on it.