Coagulation Activation in Children with Sickle Cell Disease Is Associated with Cerebral Small Vessel Vasculopathy

Raffaella Colombatti, Emiliano De Bon, Antonella Bertomoro, Alessandra Casonato, Elena Pontara, Elisabetta Omenetto, Graziella Saggiorato, Agostino Steffan, Tamara Damian, Giuseppe Cella, Simone Teso, Renzo Manara, Patrizia Rampazzo, Giorgio Meneghetti, Giuseppe Basso, Maria Teresa Sartori, Laura Sainati

Research output: Contribution to journalArticlepeer-review


Background:Thrombotic complications in Sickle Cell Disease (SCD) arise since infancy, but the role of the coagulation system in children has been poorly explored. To determine its role in the development of clinical complications in childhood we measured coagulation and endothelial parameters in children with SCD at steady state.Methods:Markers of thrombin generation, fibrin dissolution and endothelial activation were evaluated in 38 children with SS-Sβ°, 6 with SC disease and 50 age and blood group matched controls. Coagulation variables were correlated with markers of hemolysis and inflammation, with the presence of cerebral and lung vasculopathy and with the frequency of clinical complications.Results:SS-Sβ° patients presented higher levels of factor VIII, von Willebrand factor antigen (VWF:Ag) and collagen binding activity, tissue plasminogen activator antigen (t-PA:Ag), D-dimer, p-selectin, prothrombin fragment1+2 (F1+2) and lower ADAMTS-13:activity/VWF:Ag (p2.5m/sec.Conclusions:SS-Sβ° disease is associated with extensive activation of the coagulation system at steady state since young age. ADAMTS-13 and t-PA:Ag are involved in the development of cerebral silent infarcts.

Original languageEnglish
Article numbere78801
JournalPLoS One
Issue number10
Publication statusPublished - Oct 25 2013

ASJC Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine(all)


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