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血清铁蛋白不是判断重型地中海贫血患者造血干细胞移植后铁过载的可靠指标。

Serum ferritin is not a reliable predictor to determine iron overload in thalassemia major patients post-hematopoietic stem cell transplantation.

机构信息

Department for Children and Adolescents, Division for Stem Cell Transplantation and Immunology, University Hospital Frankfurt, Frankfurt am Main, Germany.

Department of Pediatrics and Adolescent Medicine, University Medical Center, Ulm, Germany.

出版信息

Eur J Haematol. 2018 Dec;101(6):791-797. doi: 10.1111/ejh.13169. Epub 2018 Oct 25.

DOI:10.1111/ejh.13169
PMID:30187571
Abstract

OBJECTIVE

Iron overload (IO) in transfusion-dependent anemia persists after hematopoietic stem cell transplantation (HSCT) and can cause long-term organ damage. In many studies, the diagnosis of IO before and after HSCT is based on serum ferritin (SF) levels rather than on assessment of liver iron concentration (LIC) by MRI or SQUID.

METHOD

In a retrospective multicenter study, we analyzed the concordance for indication of iron depletion therapy and correlation between LIC and SF of 36 thalassemia patients after HSCT. LIC was determined either by MRI-R2 (FerriScan®) or SQUID.

RESULTS

The concordance between LIC and SF varies over time after transplant (P = 0.011). The correlation between SF and LIC was strong in the first year (Spearman's rho 0.75; P < 0.001). In agreement, the concordance between SF and LIC concerning indication for treatment was close to 1 with an overall error rate ca. of 10%. In particular in the first year after HSCT, SF underestimates the degree of iron overload. However, in the longitudinal analysis since the second year post-HSCT onward no association was found between LIC and SF (P = 0.217). Furthermore, in the second year after HSCT, the overall error rate was 35%, whereas in the 3rd, 4th, and >4th year, it was 58%, 60%, and 25%, respectively.

CONCLUSIONS

Our data suggest serum ferritin is not a reliable predictor to determine iron overload in thalassemia patients after HSCT.

摘要

目的

在造血干细胞移植(HSCT)后,输血依赖型贫血患者仍存在铁过载(IO),并可能导致长期器官损伤。在许多研究中,HSCT 前后 IO 的诊断基于血清铁蛋白(SF)水平,而不是通过 MRI 或 SQUID 评估肝脏铁浓度(LIC)。

方法

在一项回顾性多中心研究中,我们分析了 36 例 HSCT 后地中海贫血患者的铁耗竭治疗指征的一致性以及 LIC 与 SF 之间的相关性。LIC 通过 MRI-R2(FerriScan®)或 SQUID 确定。

结果

移植后 LIC 与 SF 的一致性随时间而变化(P=0.011)。SF 与 LIC 在第一年的相关性较强(Spearman 秩相关系数 0.75;P<0.001)。相应地,SF 与 LIC 在治疗指征方面的一致性接近 1,总误差率约为 10%。特别是在 HSCT 后第一年,SF 低估了铁过载的程度。然而,在纵向分析中,自 HSCT 后第二年起,LIC 与 SF 之间未发现相关性(P=0.217)。此外,在 HSCT 后第二年,总误差率为 35%,而在第三、第四和>第四年,分别为 58%、60%和 25%。

结论

我们的数据表明,血清铁蛋白不是 HSCT 后地中海贫血患者确定铁过载的可靠预测指标。

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