目的 联合血清铁蛋白、心脏及肝脏磁共振T2-star(T2*)评估重型β-地中海贫血(β-TM)患者的心脏、肝脏铁超负荷的临床意义.方法 南方医科大学南方医院输血+去铁治疗的β-TM中选择28例≥10岁,血清铁蛋白＞1000 μg/L的患儿,2010～2011年分批赴香港进行心脏T2*、肝脏T2*检测,检测结果分别与年龄、血清铁蛋白、左心室射血分数(LVEF)进行比较.结果 14例(50％)发生心肌铁超负荷,其中心脏T2*＜10 ms(重度)7例,10～14 ms(中度)2例,15～20 ms(轻度)5例；肝脏铁过载28例(100％),其中2.7～6.3 ms(轻度)2例,1.4～2.7 ms(中度)7例,＜1.4 ms(重度)19例；发生LVEF下降共2例(7.14％),其中1例患者重度心肌铁超负荷；肝脏T2*与心脏T2*呈正相关(r=0.378,P=0.047),心脏T2*与血清铁蛋白呈负相关(r=-0.479,P=0.01),年龄与血清铁蛋白、LVEF、心脏T2*、肝脏T2*均不相关.结论 磁共振成像(T2*)是检测β-TM患者输血所致心脏、肝脏铁超负荷的有效、无创的手段,联合血清铁蛋白可作为评估机体重要脏器铁过载的主要诊断指标.%Objective To assess the value of magnetic resonance imaging T2* tests in the detection of myocardial and liver iron overload in patients with β-thalassemia major (p-TM). Methods From 2010 to 2011, 28 β-TM patients over 10 years old under blood transfusion therapy and chelation care with serum ferritin (SF)>1000 μg/L underwent myocardial and liver MRI T2* tests on a voluntary basis. The results were analyzed in relation with age, SF, and left ventricular ejection fraction (LVEF). Results Fourteen out of the 28 cases (50%) were found to have myocardial iron overload, including 7 severe cases, 2 moderate cases, and 5 mild cases. All the 28 cases had liver iron overload, including 2 mild cases, 7 moderate cases, and 19 severe cases. Two out of the 28 cases had lowered LVEF (7.14%), and one of them had severe myocardial iron overload. There was a negative correlation between myocardial MRI T2* and SF (r=-0.479, P=0.01). Myocardial MRI T2* was positively correlated with liver MRI T2* (r=0.378, P=0.047). Age was not significantly correlated with SF, LVEF, or liver MRI T2*. Conclusion Magnetic resonance imaging (T2*) detection is an effective and non-invasive means for detecting myocardial and liver iron overload in patients with β-thalassemia major receiving blood transfusion. T2* combined with SF is the main diagnostic indicator to assess iron overload in the vital organs.
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