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作 者:阳文景 廖建云[1] 温建芸[1] 阮永胜[1] 陈丽白[1] 何岳林[1] 李春富[1] 吴学东[1] Yang Wen-jing;Liao Jian-yun;Wen Jian-yun;Ruan Yong-sheng;Chen Li-bai;He Yue-lin;Li Chun-fu;Wu Xue-dong(Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong Province, China;Department of Pediatrics, Liuzhou Municipal Liutie Central Hospital, Liuzhou 545007, Guangxi Zhuang Autonomous Region, China)
机构地区:[1]南方医科大学附属南方医院儿科,广东省广州市510515 [2]柳州市柳铁中心医院儿科,广西壮族自治区柳州市545007
出 处:《中国组织工程研究》2017年第33期5293-5298,共6页Chinese Journal of Tissue Engineering Research
基 金:广东省科技计划项目(2014A020212186);南方医院院长基金(2014B012)~~
摘 要:背景:大部分的重型β-地中海贫血患儿都存在铁过载的情况,而铁过载可对造血干细胞移植产生不利影响。目的:利用MRI T2*检测肝脏及心脏铁过载情况,从而评估肝脏、心脏铁过载对重型β-地中海贫血患儿全相合异基因造血干细胞移植的影响。方法:对81例全相合异基因造血干细胞移植、年龄>3岁,能配合MRI检测的重型β-地中海贫血患儿,进行肝脏及心脏MRI T2*检测。根据检测结果计算出肝脏及心脏铁浓度,作为评价肝心铁过载的指标,并将肝脏、心脏铁浓度与患儿血清铁蛋白、造血重建时间、死亡率、植入率、移植后常见并发症的发病率,如移植物抗宿主病、感染、免疫性溶血、全血细胞减少、肝静脉阻塞病、败血症进行相关因素分析。结果与结论:肝脏铁浓度与血红蛋白植入时间呈正相关(r=0.229,P=0.043),心脏铁浓度与死亡率呈正相关(r=0.266,P=0.017),血清铁蛋白与植入率呈负相关(r=-0.289,P=0.009),血清铁蛋白与败血症发病率呈正相关(r=0.251,P=0.024),血清铁蛋白与全血细胞减少发病率呈正相关(r=0.276,P=0.013)。由此可见,铁过载可对重型β-地中海贫血患儿全相合异基因造血干细胞移植产生不利影响。因此,对行造血干细胞移植的β-地中海贫血患儿综合进行血清铁蛋白、肝脏及心脏铁过载的评估非常必要。BACKGROUND:The majority of children withβ-thalassemia major have iron overload,and iron overload may have negative effects on hematopoietic stem cell transplantation.OBJECTIVE:To assess the effects of liver and cardiac iron overload detected by magnetic resonance imaging(MRI)T2*on HLA-identical allogeneic hematopoietic stem cell transplantation in children withβ-thalassemia major.METHODS:Eighty-one children withβ-thalassemia major who were over3years of age and could cooperate with MRI detection were subjected to liver and heart MRI T2*tests before or after HLA-identical allogeneic hematopoietic stem cell transplantation.According to the test results,we calculated the liver and cardiac iron content,defined as an indicator of liver and heart iron overload.Then,there was a correlation analysis between the liver and cardiac iron content and serum ferritin,time of hematopoietic reconstitution,mortality rate,implantation rate and the morbidity of transplantation related complications,such as graft-versus-host disease,infections,autoimmune hemolysis,pancytopenia,hepatic veno-occlusive disease,septicemia.RESULTS AND CONCLUSION:The liver iron content was positively correlated with the time of hemoglobin implantation(r=0.229,P=0.043),and the cardiac iron content were positively correlated with the mortality rate(r=0.266,P=0.017);the serum ferritin level was negatively correlated with the implantation rate(r=-0.289,P=0.009),and positively correlated with the morbidity of septicemia(r=0.251,P=0.024)and pancytopenia(r=0.276,P=0.013).Therefore,iron overload exerts negative effects on HLA-identical allogeneic hematopoietic stem cell transplantation inβ-thalassemia major children,and it is necessary to detect serum ferritin level and assess liver and cardiac iron overload before cell transplantation.
关 键 词:干细胞 移植 重型Β地中海贫血 铁过载 造血干细胞移植 核磁共振T2* 肝铁浓度 心铁浓度 血清铁蛋白 败血症 植入率 血红蛋白植入时间
分 类 号:R394.2[医药卫生—医学遗传学]
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