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湖南首例儿童重型地中海贫血造血干细胞移植顺利出仓

2017-08-22 17:16:50   央广网

央广网郴州8月22日消息(记者傅蕾 通讯员胡国华 刘振)8月22日,湖南郴州市第一人民医院发布,湖南省首例儿童重型地中海贫血造血干细胞移植患者佳佳(化名)顺利出仓

6年前,1岁多的男孩佳佳因“面色苍白”被确诊为重型β地中海贫血(β双重杂合子),需要依靠输血(输注红细胞)维持生命。

家长想给佳佳做造血干细胞移植(原来俗称骨髓移植),佳佳2岁时到广州某医院做了移植前配型,很幸运的是佳佳跟他姐姐的HLA配型是全相合的,但是昂贵的移植费用让佳佳家长望而却步。

所以佳佳一直定期输血,输血前血红蛋白维持在(40-50)g/L,但是输血的次数越来越频繁,从开始的一个半月一次,到一个月,再到二十天一次。

因为长期输血,佳佳肝脾越来越大,到移植前肝脾肿大已超过了肚脐,慢性铁沉积也越来越重,这种情况需要规范的去铁治疗,因经济原因家长2年前开始只能间断使用去铁酮、恩瑞格去铁治疗,最后铁蛋白已达4000mg/L。

佳佳每年输血加上去铁治疗的费用是4-5万,唯一能解决的办法就是做造血干细胞移植。

郴州市第一人民医院北院(儿童医院)经过详细的术前评估,儿童血液科主任阳睿决定给佳佳做移植。佳佳于一个月前入院,开始进入造血干细胞移植程序。供者是佳佳的二姐,今年10岁。

佳佳先入仓行预处理化疗,然后分别予骨髓造血干细胞、外周血造血干细胞输注治疗。输注骨髓造血干细胞后佳佳出现一次抽搐,予以积极处理后缓解,后未再抽搐。

移植8天后,患者出现发热,口腔黏膜有溃疡,考虑植入综合征,予抑制免疫及抗感染治疗,患者发热好转,体温平稳。

18天后,患者中性粒细胞连续3天大于500,提示干细胞植活,也就是造血干细胞移植成功,从移植层流仓转入普通病房。

郴州市第一人民医院北院(儿童医院)儿童血液科主任阳睿介绍,湖南省首例儿童重型地中海贫血造血干细胞移植佳佳顺利出仓,标志着郴州市第一人民医院北院(儿童医院)儿童血液内科已经处于湖南省儿科造血干细胞移植领先水平。

这大大的提高了郴州市第一人民医院北院(儿童医院)的专业地位和核心竞争力,使解决周边地区许多重大血液疾病儿童的生存问题并使这些患者获得长期生存变得可能。

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