Background: Hydroxyurea (HU) is the most successful pharmacological therapy with improvement in the quality of life of patients with sickle cell disease (SCD). Studies have reported hemostatic disorders in SCD, such as elevated plasma levels of plasminogen activator inhibitor type 1 (PAI-1) and D-dimer (D-Di).

Aims: To assess the association between the use of HU and plasma levels of PAI-1 and D-Di hemostatic markers in patients with SCD.

Methods: Cross-sectional study enrolling 40 patients with SCD from the Hemonúcleo Regional de Divinópolis / MG, Brazil, aged over 18 years, using (n = 16) or not (n = 24) HU, and 40 healthy controls matched by gender and age. The hemostatic markers PAI-1 and D-Di were determined by ELISA, whose results were expressed as mean ± SD and median (interquartile range), respectively, and analyzed by t Student´s, Mann-Whitney, ANOVA and Kruskal-Wallis tests, with significance level < 0.05. The study received approval from the local Ethics Committee.

Results: Plasma levels of PAI-1 and D-Di were higher in patients with SCD [113.64 ± 24.89 ng/mL and 611.07 (402.82-155.25) ng/mL, respectively] than in the controls [65.00 ± 25.24ng/mL and 74.77 (49.55-122.65) ng/mL, respectively] (p < 0.001 for both). However, there was no significant difference in the plasma levels of these markers between patients with SCD using or not HU.

Conclusions: The use of HU was not able to favorably modify the hemostatic changes resulting from SCD, such as hypofibrinolysis and hypercoagulability, conditions related to the increase in plasma levels of PAI-1 and D-Di, respectively.
Ackowledgement: We are very grateful to the staff of Hemonúcleo Regional de Divinópolis, MG, Brazil, for the valuable help, as well as to CNPq, FAPEMIG and CAPES for the financial support.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/evaluation-of-hemostatic-markers-in-patients-with-sickle-cell-disease-using-or-not-hydroxyurea/