Abstract Number: PB0746
Meeting: ISTH 2020 Congress
Background: Sickle cell disease (SCD) is frequently referred to as a hypercoagulable state. Hydroxyurea (HU) is known to decrease the frequency of vaso-occlusive complications and need for blood transfusions in severely affected individuals.
Aims: Our aim wasto assess the effect of HU on markers of fibrinolysis (D-dimer) and endothelial activation (soluble vascular cell adhesion molecule-1 [soluble VCAM-1]) in patients with SCD in their non-crisis, “steady state.”
Methods: Patient’s ≤15 years of age, with documented HbSS or HbSβ-thalassemia, eligible for treatment with HU were studied in this prospective, observational study.
Results: Seventy-five patients with SCD were enrolled (females: 45 [60%]), with a median age of 13 years (IQR: 3). Following 6 months of HU, median values for WBC count (9.02 x 109/L vs. 7.4 x 109/L, p< 0.001), APTT (33 sec vs. 24 sec p< 0.000), D-dimer (1243.2 ng/mL vs. 802.9 ng/mL,
p< 0.000), lactate dehydrogenase (744 U/L vs. 562 U/L, p< 0.002), soluble VCAM-1 (544.9 ng/mL vs. 492 ng/mL, p< 0.003), platelet count (250 109/L vs. 196 109/L, p< 0.002), and retic count (11.7% vs. 5.4%, p< 0.000) were significantly lower than baseline values, while the mean corpuscular volume (74 fL vs. 76 fL, p< 0.000) were higher than the baseline value. No significant differences from baseline was observed in the median values for hemoglobin
(9.1 g/dL vs. 10.5 g/dL, p< 0.72) following 6 months of HU therapy.
Conclusions: In this study of children from Pakistan, we find that treatment with HU for up to 6 months resulted in decreased levels of D-dimer and VCAM-1. With the increasing evidence that coagulation activation contributes to SCD pathophysiology, the clinical benefit of HU may be due, in part, to decreased coagulation activation.
To cite this abstract in AMA style:Borhany M, Abid M, Zafar S, Shamsi T. To Evaluate the Effect of Hydroxyurea on Blood Coagulation and Endothelial Activation in Sickle Cell Disease [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/to-evaluate-the-effect-of-hydroxyurea-on-blood-coagulation-and-endothelial-activation-in-sickle-cell-disease/. Accessed September 29, 2023.
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