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与定期输血的地中海贫血和镰状细胞病患者相比,血清铁蛋白低估了非输血依赖型地中海贫血患者的肝脏铁浓度。

Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients.

作者信息

Pakbaz Zahra, Fischer Roland, Fung Ellen, Nielsen Peter, Harmatz Paul, Vichinsky Elliott

机构信息

Children's Hospital & Research Center Oakland, Oakland, California 94609, USA, and University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

出版信息

Pediatr Blood Cancer. 2007 Sep;49(3):329-32. doi: 10.1002/pbc.21275.

Abstract

Serum ferritin (SF) and liver iron concentration (LIC), as measured by SQUID biosusceptometry, were assessed in a convenience sample of transfusion independent thalassemia patients (nTx-Thal, n=26), regularly transfused thalassemia (Tx-Thal, n=89), or sickle cell patients (SCD, n=45) to investigate the severity of iron overload and the relationship between SF and LIC in nTx-Thal compared to SCD and Tx-Thal. SF correlated with LIC (RS=0.53, P<0.001), but was found to be a poor predictor for LIC. SF was significantly lower (P<0.001) in nTx-Thal patients than in other groups, despite similar LIC values. The SF-to-LIC ratio was significantly lower in nTx-Thal compared to Tx-Thal and SCD patients (median of 0.32, 0.87, and 1.2, respectively: P<0.001). Due to underestimation of LIC by ferritin levels, chelation treatment may be delayed or misdirected in patients with thalassemia intermedia.

摘要

通过超导量子干涉装置生物磁测量法测量血清铁蛋白(SF)和肝脏铁浓度(LIC),在一组方便样本中对无需输血的地中海贫血患者(nTx-Thal,n = 26)、定期输血的地中海贫血患者(Tx-Thal,n = 89)或镰状细胞病患者(SCD,n = 45)进行评估,以研究铁过载的严重程度以及与SCD和Tx-Thal相比nTx-Thal中SF和LIC之间的关系。SF与LIC相关(RS = 0.53,P < 0.001),但被发现是LIC的一个较差预测指标。尽管LIC值相似,但nTx-Thal患者的SF显著更低(P < 0.001)。与Tx-Thal和SCD患者相比,nTx-Thal患者的SF与LIC之比显著更低(中位数分别为0.32、0.87和1.2:P < 0.001)。由于铁蛋白水平对LIC的低估,中间型地中海贫血患者的螯合治疗可能会延迟或出现方向错误。

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