CAJ | 학술논문

目的:总结分析动员后的同胞骨髓联合脐血混合移植治疗儿童重型β-地中海贫血的效果.方法;南方医科大学附属南方医院儿科2005-01/2009-03行同胞骨髓+脐血混合移植的重型β-地中海贫血患儿8例,男5例,女3例,年龄4.0～7.5岁,按Pesaro标准进行地中海贫血临床分度,Ⅰ～Ⅱ度7例,Ⅲ度1例.供者年龄1～4岁,预处理前4 d开始予以粒细胞集落刺激因子10 μg/(kg·d),共5 d,第5天抽取骨髓以及输注脐带血至患儿体内.结果:所有患儿均在移植后4周内恢复造血.8例患儿中有7例发生感染,4例患儿出现Ⅰ度肝静脉闭塞,2例患儿出现Ⅰ度急性移植物抗宿主病,1例患儿发生局限型慢性移植物抗宿主病.移植后32 d因肺部感染和心脏衰竭1例死亡,7例存活.结论:动员后的同胞骨髓联合脐血混合移植治疗儿童重型β-地中海贫血安全可靠,初步结果满意,但移植后存在一定的并发症需高度重视.
목적:총결분석동원후적동포골수연합제혈혼합이식치료인동중형β-지중해빈혈적효과.방법;남방의과대학부속남방의원인과2005-01/2009-03행동포골수+제혈혼합이식적중형β-지중해빈혈환인8례,남5례,녀3례,년령4.0～7.5세,안Pesaro표준진행지중해빈혈림상분도,Ⅰ～Ⅱ도7례,Ⅲ도1례.공자년령1～4세,예처리전4 d개시여이립세포집락자격인자10 μg/(kg·d),공5 d,제5천추취골수이급수주제대혈지환인체내.결과:소유환인균재이식후4주내회복조혈.8례환인중유7례발생감염,4례환인출현Ⅰ도간정맥폐새,2례환인출현Ⅰ도급성이식물항숙주병,1례환인발생국한형만성이식물항숙주병.이식후32 d인폐부감염화심장쇠갈1례사망,7례존활.결론:동원후적동포골수연합제혈혼합이식치료인동중형β-지중해빈혈안전가고,초보결과만의,단이식후존재일정적병발증수고도중시.
OBJECTIVE: To investigate the curative effect of combined transplantation of bone marrow and umbilical cord blood of same sibling in children with β-thalassemia major.METHODS: Eight thalassemia major patients undergoing combined transplantation of bone marrow and umbilical cord blood of same sibling aged from 4.0 to 7.5 years, 5 boys and 3 girls, were recruited at the Department of Pediatrics, Nanfang Hospital,Southem Medical University from January 2005 to March 2009. The patients were classified into three classes according to Pesarothalassamia classification, class Ⅰ to class Ⅱ 7 cases and class Ⅲ 1 case. Donors ranged 1-4 years received 10 μg/kg per day of subcutaneous granulocyte colony-stimulating factor (G-CSF) for 5 consecutive days. Bone marrow was harvested on the fifth day. Bone marrow and umbilical cord blood of the same sibling then were transfused into the patient.RESULTS: Recovery of hematopoiesis was gained in all patients 4 weeks following transplantation. Seven patients suffered from infection of different degree. Four patients developed mild venous occlusive disease. Two patients developed grade Ⅰ acute graft-versus-host disease (GVHD), and one developed grade Ⅰ chronic GVHD. Seven patients were alive and one died of pulmonary infection and heart failure 32 days following transplantation.CONCLUSION: Combined transplantation of granulocyte colony-stimulating factor primed bone marrow and umbilical cord blood of same sibling in children with β-thalassemia major is safe and effective with promising results. However, complications should be paid high attention following transplantation.