Management of children with bleeding disorders

Abstract

Bleeding and easy bruising in children may be due to one of the many inherited or acquired disorders of hemostasis. An understanding of normal hemostasis is essential for evaluation of a child with abnormal bleeding. A complex interaction between vascular endothelium, von Willebrand factor and procoagulant protein Factor I to XIII results in formation of a fibrin clot at the site of injury and bleeding stops. Conditions causing quantitative or qualitative defects of platelets, von Willebrand factor or various procoagulant protein will result in abnormal bleeding. Patients with hemophilia experience more severe bleeding and are diagnosed early in life. von Willebrand disease on the contrary has a variable presentation and therefore the diagnosis is delayed in some cases. Morbidity and mortality due to bleeding may be reduced with appropriate management in the majority of patients with bleeding disorders. A systematic approach to a patient with bleeding is discussed. Management of hemophilia and von Willebrand disease is also reviewed.