Department of Pediatric Hematology-Oncology, "Aghia Sophia" Children's Hospital, Athens, Greece.
First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Eur J Haematol. 2018 Jun;100(6):630-635. doi: 10.1111/ejh.13061. Epub 2018 Apr 25.
Levels of the angiogenic cytokines placental growth factor (PlGF) and soluble Fms-like tyrosine kinase-1 (sFlt-1) and the angiogenic balance, expressed by sFlt-1/PlGF ratio, are perturbed in sickle-cell disease and iron overload, but they have not been evaluated in non-transfusion-dependent thalassemia (NTDT).
We measured levels of PlGF, sFlt-1 and vWF:antigen in patients with NTDT of beta-thalassemia genotype, and correlated them with erythrocytic indices and markers of iron overload, inflammation, and tissue hypoxia. Thirty-four NTDT patients with mean hemoglobin level of 8.4 g/dL were included in the study along with 20 apparently healthy individuals who served as controls.
Ferritin, LDH, and hs-CRP were higher in patients as compared to controls. We found significant differences between patients and controls in regard to levels of PlGF (52.2 vs 17.2 pg/mL, P < .001), sFlt-1/PlGF (2 vs 4.7, P < .001), and vWF:antigen (88 vs 77.1 IU/dL, P < .01). There was a strong correlation of ferritin with PlGF (r = .653, P < .001) and with vWF:antigen (r = .503, P = .003).
In this study, we demonstrated an association between increased PlGF and iron overload and the degree of tissue hypoxia in patients with NTDT. High vWF:antigen expressing endothelial damage may be associated with specific NTDT comorbidities.
在镰状细胞病和铁过载中，血管生成细胞因子胎盘生长因子 (PlGF) 和可溶性 Fms 样酪氨酸激酶-1 (sFlt-1) 的水平以及血管生成平衡（以 sFlt-1/PlGF 比值表示）受到干扰，但在非输血依赖型地中海贫血 (NTDT) 中尚未进行评估。
我们测量了β地中海贫血基因型 NTDT 患者的 PlGF、sFlt-1 和 vWF:抗原水平，并将其与红细胞指数和铁过载、炎症和组织缺氧标志物相关联。研究纳入了 34 名平均血红蛋白水平为 8.4 g/dL 的 NTDT 患者，以及 20 名作为对照的健康个体。
与对照组相比，患者的铁蛋白、乳酸脱氢酶和高敏 C 反应蛋白水平更高。我们发现患者与对照组在 PlGF（52.2 对 17.2 pg/mL，P <.001）、sFlt-1/PlGF（2 对 4.7，P <.001）和 vWF:抗原（88 对 77.1 IU/dL，P <.01）水平方面存在显著差异。铁蛋白与 PlGF（r =.653，P <.001）和 vWF:抗原（r =.503，P =.003）呈强相关。
在这项研究中，我们证明了 NTDT 患者中 PlGF 增加与铁过载和组织缺氧程度之间存在关联。高 vWF:抗原表达的内皮损伤可能与特定的 NTDT 合并症相关。
