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β 中间型地中海贫血非输血依赖患者的生长分化因子 15 水平较高,与临床严重程度相关。

Levels of growth differentiation factor-15 are high and correlate with clinical severity in transfusion-independent patients with β thalassemia intermedia.

机构信息

Department of Internal Medicine, Division of Hematology and Oncology, American University of Beirut Medical Center, Lebanon.

出版信息

Blood Cells Mol Dis. 2011 Dec 15;47(4):232-4. doi: 10.1016/j.bcmd.2011.07.005. Epub 2011 Aug 23.

DOI:10.1016/j.bcmd.2011.07.005
PMID:21865063
Abstract

Transfusion-independent patients with β thalassemia intermedia (TI) experience a variety of clinical complications attributed to the underlying ineffective erythropoiesis and subsequent anemia, hemolysis, and iron overload. Growth differentiation factor-15 (GDF-15) was recently investigated as a marker of ineffective erythropoiesis in several anemias. In this work, we evaluated GDF-15 levels in 55 patients with TI. The mean GDF-15 level was 25,197.8±16,208.9pg/ml which is lower than values reported for patients with thalassemia major, yet considerably higher than those reported in patients with other congenital and acquired anemias. GDF-15 levels were significantly higher in splenectomized compared to non-splenectomized patients and correlated with anemia, markers of iron overload, and a pre-defined clinical severity score. Further studies are needed to determine the practical utility of GDF-15 measurement and its potential to reflect the severity of the clinical course in TI patients.

摘要

无脾症状β地中海贫血中间型(TI)患者经历各种临床并发症归因于基础无效红细胞生成和随后的贫血、溶血和铁过载。生长分化因子 15(GDF-15)最近在几种贫血症中被作为无效红细胞生成的标志物进行了研究。在这项工作中,我们评估了 55 例 TI 患者的 GDF-15 水平。GDF-15 的平均水平为 25197.8±16208.9pg/ml,低于重型地中海贫血患者的报道值,但明显高于其他先天性和获得性贫血患者的报道值。脾切除患者的 GDF-15 水平明显高于非脾切除患者,且与贫血、铁过载标志物和预先定义的临床严重程度评分相关。需要进一步的研究来确定 GDF-15 测量的实际效用及其在反映 TI 患者临床病程严重程度方面的潜力。

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