Pakbaz Zahra, Fischer Roland, Fung Ellen, Nielsen Peter, Harmatz Paul, Vichinsky Elliott
Children's Hospital & Research Center Oakland, Oakland, California 94609, USA, and University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Pediatr Blood Cancer. 2007 Sep;49(3):329-32. doi: 10.1002/pbc.21275.
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.
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。