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Enhanced thrombin generation in children with sickle cell disease

M Peters1, B E Plaat, H ten Cate

  • 1Emma Kinderziekenhuis/Children's Academic Medical Center, Amsterdam, The Netherlands.

Thrombosis and Haemostasis
|February 1, 1994
PubMed

Insights

Children with sickle cell disease (SCD) show increased thrombin generation, a marker of blood clot formation. This may be linked to lower levels of protein C and protein S, crucial coagulation inhibitors in pediatric SCD patients.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Thrombosis Research

Background:

  • Sickle cell disease (SCD) is an inherited blood disorder with significant childhood morbidity.
  • Vascular occlusion in SCD may involve increased coagulation system activity.
  • Previous studies on coagulation in SCD primarily focused on adult populations.

Purpose of the Study:

  • To investigate coagulation system activity in children with sickle cell disease.
  • To assess markers of thrombin generation and coagulation inhibitors in pediatric SCD patients.

Main Methods:

  • Prospective study comparing 16 homozygous SCD patients with 16 age-matched controls.
  • Measurement of prothrombin fragment F1+2 and thrombin-antithrombin III (TAT) complexes.
  • Assessment of protein C activity, total and free protein S, and antithrombin III (AT III) levels.

Main Results:

  • SCD patients exhibited significantly elevated plasma concentrations of F1+2 and TAT complexes.
  • Significantly reduced levels of protein C activity and total/free protein S were observed in SCD patients.
  • Plasma AT III levels did not differ between SCD patients and controls.

Conclusions:

  • Children with SCD demonstrate evidence of enhanced thrombin generation.
  • Reduced levels of protein C and protein S may contribute to the observed hypercoagulable state in pediatric SCD.
  • Further research is needed to establish the clinical significance of this coagulation imbalance in childhood SCD.

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