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原发性骨髓纤维化患者转铁蛋白饱和度低的预后意义

Prognostic implications of low transferrin saturation in patients with primary myelofibrosis.

作者信息

Lucijanic Marko, Prka Zeljko, Pejsa Vlatko, Stoos-Veic Tajana, Lucijanic Jelena, Kusec Rajko

机构信息

Hematology Department, University Hospital Dubrava, Av. Gojka Suska 6, 10000 Zagreb, Croatia.

School of Medicine, University of Zagreb, Salata 3, 10000 Zagreb, Croatia.

出版信息

Leuk Res. 2018 Mar;66:89-95. doi: 10.1016/j.leukres.2018.01.017. Epub 2018 Feb 3.

DOI:10.1016/j.leukres.2018.01.017
PMID:29407589
Abstract

OBJECTIVES

Transferrin saturation (TSAT) 20% or less is considered to represent functional iron deficiency in the context of malignant disease, phenomenon mediated through inflammatory changes of iron homeostasis. We aimed to investigate clinical and prognostic significance of low TSAT in patients with primary (PMF) and secondary myelofibrosis (SMF), malignant diseases characterized by strong inflammatory milieu.

METHODS

We retrospectively analyzed 87 patients with myelofibrosis and compared TSAT with disease specific parameters.

RESULTS

One-third of patients had TSAT ≤20%. Lower TSAT was significantly associated with Janus-kinase-2 (JAK2) mutation (P = 0.007), transfusion independency (P = 0.003), higher platelets (P = 0.004), lower mean-corpuscular-volume (P < 0.001), lower ferritin (P < 0.001), higher absolute-neutrophil-count (P = 0.027), lower absolute-lymphocyte-count (P = 0.041) and lower albumin (P = 0.018). PMF patients presenting with low TSAT (≤20%) experienced significantly shorter overall-survival (OS) (HR = 2.43; P = 0.017), whereas TSAT did not affect OS of SMF patients (HR = 1.48; P = 0.623). Low TSAT remained significantly associated with inferior OS in PMF in a series of multivariate Cox regression models comparing its properties to anemia, transfusion dependency, ferritin and Dynamic-International-Prognostic-System (DIPSS).

CONCLUSIONS

Low TSAT has detrimental effect on survival of PMF patients. This effect is independent of anemia and of ferritin levels that seem to be better at representing iron overload in PMF patients.

摘要

目的

转铁蛋白饱和度(TSAT)≤20%被认为在恶性疾病中代表功能性缺铁,这种现象是由铁稳态的炎症变化介导的。我们旨在研究低TSAT在原发性骨髓纤维化(PMF)和继发性骨髓纤维化(SMF)患者中的临床和预后意义,这两种恶性疾病的特点是炎症环境强烈。

方法

我们回顾性分析了87例骨髓纤维化患者,并将TSAT与疾病特异性参数进行比较。

结果

三分之一的患者TSAT≤20%。较低的TSAT与Janus激酶2(JAK2)突变显著相关(P = 0.007)、不依赖输血(P = 0.003)、血小板较高(P = 0.004)、平均红细胞体积较低(P < 0.001)、铁蛋白较低(P < 0.001)、绝对中性粒细胞计数较高(P = 0.027)、绝对淋巴细胞计数较低(P = 0.041)和白蛋白较低(P = 0.018)。TSAT低(≤20%)的PMF患者总生存期(OS)明显较短(HR = 2.43;P = 0.017),而TSAT不影响SMF患者的OS(HR = 1.48;P = 0.623)。在一系列多变量Cox回归模型中,将低TSAT与贫血、输血依赖性、铁蛋白和动态国际预后系统(DIPSS)的特性进行比较时,低TSAT在PMF中仍与较差的OS显著相关。

结论

低TSAT对PMF患者的生存有不利影响。这种影响独立于贫血和铁蛋白水平,铁蛋白水平似乎能更好地反映PMF患者的铁过载情况。

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