摘要:Objective To explore the relationship of long-term blood transfusion, iron chelation therapy with iron overload in the patients with beta-thalassaemia major. Methods The serum ferritin (SF) , liver function, renal function, myocardial enzyme, ultrasonograph of liver and spleen, blood glucose and urine glucose were detected in the patients with beta-thalassaemia major in "Friends of thalassemia" and "Service team for thalassemia in Shenzhen" with regular long-term blood transfusion and iron chelation therapy in three months. Left ventricular ejection fraction (LVEF) , myocardial, liver, pancreas and pituitary MR imaging T2* were performed on 51 patients of them. The51 patients were divided into 3 groups. 10 cases with sufficient dose DFO and sufficient dose DFP iron chelation treatment group (sufficient dose joint group) ; 31 cases with insufficient DFO and DFP iron chelation treatment group (insufficient joint group) ; 10 cases with sufficient dose DFX iron chelation treatment group (DFX group). Results There was no difference in myocardial T2*, pituitary T2*, LVEF in every group. (P > 0.05). Liver T2* in sufficient dose joint group was higher than that in insufficient joint group (P < 0.05). Liver T2*, pancreas T2*, and pituitary T2*in DFX group was significantly higherthan that in the other two groups (P < 0.05). SF in sufficient dose joint group was significantly lower than that in insufficient joint group (P < 0.05) , SF in DFX group was significantly lower than that inthe other two groups (P < 0.05). Liver T2* and pancreas T2*in C group were significantly lower than those in A and B groups (P < 0.05).There was myocardial iron overload in 11 of 5lcases (21.6%), and there was liver iron overload in 43 of 51 cases (84.3%). SF had no correlation with myocardial T2*(r = 0.254, P > 0.05), and there was moderate negative correlation with liver T2*(r = 0.558,P < 0.01). Conclusion The sufficient dose DFO with DFP treatment, and the DFX treatment can effectively reduce SF, compared with that of insufficient DFO with DFP treatment. All iron chelation therapy can alleviate myocardial iron overload.%目的 探討重型β珠蛋白生成障礙性貧血(beta-thalassaemia,簡稱β -TM)患兒長期輸血、去鐵治療與鐵過載的關系.方法 深圳市第二人民醫院2001年成立“地貧之友”與“地貧服務隊”,對β-TM患兒進行規范性的長期輸血和去鐵治療.每3個月監測血清鐵蛋白濃度(SF)、肝腎功能、心肌酶譜、心功能、心臟和肝脾B超、血糖和尿糖.2001年2月至2010年6月對其中51例患兒進行核磁共振檢測心臟T2*、左心室射血分數(LVEF)、肝臟T2*、胰腺T2*和垂體T2*.根據治療方法分為足療程去鐵胺+去鐵酮(DFO+DFP)聯合去鐵治療組(足療程聯合組)10例、不足療程DFO+DFP聯合去鐵治療組(不足療程聯合組)31例、單用足療程地拉羅司(DFX)去鐵治療組(單用DFX組)10例.根據SF質量濃度分為SF≤2000 μg/L組(A組)12例、SF-3000 μg/L組(B組)17例、SF>3000μg/L組(C組)22例.結果 各組LVEF、心臟T2*、垂體T2*值差異無統計學意義(P>0.05);足療程聯合組肝臟T2*高于不足療程聯合組(P<0.05),單用DFX組肝臟T2*、胰腺T2*、垂體T2*均高于足療程聯合組和不足療程聯合組(P<0.05).足療程聯合組SF低于不足療程聯合組,單用DFX組SF低于足療程聯合組和不足療程聯合組,差異均具有統計學意義(P<0.05).C組肝臟T2*和胰腺T2*明顯低于A組和B組,差異具有統計學意義(P<0.05).心肌鐵過載11例(21.6%),肝臟鐵過載43例(84.3%).SF與心臟T2*無相關性(r=0.254,P>0.05),與肝臟T2*呈中度負相關(r=0.558,P< 0.01).結論 足療程DFO+DFP聯合去鐵治療和單用足療程DFX去鐵治療均能有效降低血清鐵蛋白濃度,動員肝臟組織鐵,效果優于不足療程DFO+DFP聯合去鐵治療.不同去鐵方式均能減輕心臟鐵過載.