Hydroxyurea in Sickle Cell Disease: Coagulation and Activation in an Observational Study.

IF 1.7 Q2 PEDIATRICS

Clinical Medicine Insights-Pediatrics Pub Date : 2026-03-05 DOI:10.1177/11795565251403177

Munira Borhany, Hafiza Tuba Iqbal, Madiha Abid, Sidra Zafar

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Abstract

Background: Sickle cell disease (SCD) is characterized by a chronic hypercoagulable state. Hydroxyurea (HU) is known to reduce the frequency of vaso-occlusive events and transfusion requirements in affected individuals.

Objective: To assess the impact of hydroxyurea on biomarkers of coagulation (D-dimer) and endothelial activation (soluble VCAM-1) in SCD patients in Pakistan in their steady state.

Methods: A prospective observational study was conducted in patients aged ⩾ 10 years with confirmed HbSS or HbSβ-thalassemia genotypes. Biomarkers were measured at baseline and after 6 months of HU therapy.

Results: Twenty-five patients (HbSS = 15 [60%], HbSβ-thalassemia = 10 [40%]) with a median (IQR) age of 23 (16.5-27) years were enrolled. A significant decrease in D-dimer levels was observed after HU treatment: from a median of 1243 to 830 ng/mL (P = .028), reflecting a 33% reduction. Soluble VCAM-1 levels showed no statistically significant change (532.6 vs 492.9 ng/mL, P = .381). HbF increased from 20.1% (12.6-27.5) to 28% (20-39) (P < .001), with a strong positive correlation with HU treatment (r = .845).

Conclusion: This study demonstrates that HU therapy in Pakistani SCD patients significantly reduces D-dimer levels, suggesting reduced thrombotic activity. While the endothelial marker VCAM-1 showed no significant change, the rise in HbF is consistent with known HU effects.

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羟基脲在镰状细胞病中的作用：一项观察性研究中的凝血和活化。

背景：镰状细胞病（SCD）以慢性高凝状态为特征。羟基脲（HU）已知可降低受影响个体血管闭塞事件的频率和输血需求。目的：评估羟基脲对巴基斯坦SCD患者稳定状态下凝血生物标志物（d -二聚体）和内皮活化（可溶性VCAM-1）的影响。方法：在年龄小于10岁的确诊HbSS或hbbs β-地中海贫血基因型患者中进行了一项前瞻性观察性研究。在基线和HU治疗6个月后测量生物标志物。结果：纳入25例患者（HbSS = 15 [60%]， HbSS - β-地中海贫血= 10[40%]），中位（IQR）年龄为23（16.5-27）岁。HU治疗后d -二聚体水平显著下降：中位数从1243 ng/mL降至830 ng/mL (P =。028)，减少了33%。可溶性VCAM-1水平变化无统计学意义（532.6 vs 492.9 ng/mL, P = 0.381）。HbF从20.1%（12.6-27.5）增加到28% (20-39)（P r = .845）。结论：本研究表明，HU治疗巴基斯坦SCD患者可显著降低d -二聚体水平，提示血栓活性降低。虽然内皮标志物VCAM-1没有明显变化，但HbF的升高与已知的HU效应一致。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Medicine Insights-Pediatrics PEDIATRICS-

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审稿时长

8 weeks