MRI定量肝脏及心脏铁过载对地中海贫血全相合造血干细胞移植的影响

Liver and cardiac iron overload is harmful to HLA-identical hematopoietic stem cell transplantation in thalassemia children: an MRI detection

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.