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骨髓移植后地中海贫血患者的心肌和肝脏T2*磁共振评估

Myocardial and hepatic T2* magnetic resonance evaluation in ex-thalassemic patients after bone-marrow transplantation.

作者信息

Mavrogeni Sophie, Gotsis Efstathios D, Berdousi Eleni, Ladis Vasilios, Verganelakis Dimitrios, Toulas Panagiotis, Cokkinos Dennis V

机构信息

Onassis Cardiac Surgery Center, Athens, Greece.

出版信息

Int J Cardiovasc Imaging. 2007 Dec;23(6):739-45. doi: 10.1007/s10554-006-9203-7. Epub 2007 Jan 19.

Abstract

Bone marrow transplantation (BMT) is the only complete cure for b-thalassemia. Iron depletion therapy is still required to remove excess iron, accumulated before BMT. Hepatic and myocardial iron load were evaluated using T2* magnetic resonance in 8 ex-thalassemic patients after BMT, aged 19.5 +/- 4.25 years, who were in iron depletion therapy. Average hepatic T2* was 18.8 +/- 11.0 msec (4.1-35.0 msec). In 4 out of 8 patients iron overload was detected, not exceeding however 4 mg/gr dry tissue. Average heart T2* was 31.0 +/- 4.6 msec (25.6-35.2 msec), not significantly different (P = 0.18) from our age-matched normal population (33.0 +/- 4.0). Normal left ventricular ejection fraction was found in 7 out of 8 patients (mean 64.5 +/- 7.0%) with the remaining having a marginal value of 54.1%. Ferritin level before BMT was 1748 +/- 451 mug/l and dropped to 536 +/- 260 microg/l at the end of iron depletion therapy after BMT. Current ferritin level was 271 +/- 253 microg/l and although it was significant lower compared to both ferritin before BMT (P < 0.001) and after iron depletion (P < 0.001), evidence of residual hepatic iron load was identified by T2*. Hepatic and myocardial T2* magnetic resonance can be used as a more reliable index than ferritin for evaluation of iron depletion therapy in ex-thalassemic patients after BMT.

摘要

骨髓移植(BMT)是β地中海贫血唯一的彻底治愈方法。仍需要进行铁消耗疗法以清除BMT前积累的过量铁。对8名接受BMT后的β地中海贫血患者进行了T2磁共振检查,以评估肝脏和心肌的铁负荷,这些患者年龄为19.5±4.25岁,正在接受铁消耗疗法。肝脏平均T2为18.8±11.0毫秒(4.1 - 35.0毫秒)。8名患者中有4名检测到铁过载,但不超过4毫克/克干组织。心脏平均T2为31.0±4.6毫秒(25.6 - 35.2毫秒),与年龄匹配的正常人群(33.0±4.0)无显著差异(P = 0.18)。8名患者中有7名左心室射血分数正常(平均64.5±7.0%),其余患者的临界值为54.1%。BMT前铁蛋白水平为1748±451微克/升,BMT后铁消耗疗法结束时降至536±260微克/升。目前铁蛋白水平为271±253微克/升,虽然与BMT前铁蛋白(P < 0.001)和铁消耗后(P < 0.001)相比均显著降低,但通过T2仍可识别出残留肝脏铁负荷的证据。与铁蛋白相比,肝脏和心肌T2*磁共振可作为评估BMT后β地中海贫血患者铁消耗疗法更可靠的指标。

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