Hemophilia And Von Willebrand Disease In Children: Emergency Department Evaluation And Management

Abstract

Hemophilia and von Willebrand disease are the most common inherited bleeding disorders encountered in the emergency department. Evidence suggests that the management of bleeding disorders in the emergency department is currently suboptimal, and literature to guide evaluation and management in this setting is limited, though some guidelines do exist. The emergency clinician must have a high index of suspicion for new diagnoses, particularly in young patients with unprovoked bleeding and children with multiple or severe bleeds. The foundation of hemophilia treatment is urgent clotting factor replacement, with replacement goals guided by the presenting complaint. Bleeding in von Willebrand disease may be treated with products containing von Willebrand factor or with desmopressin. This review focuses on the epidemiology, pathophysiology, common presentations, evaluation strategies, and emergency management of these bleeding disorders.

Keywords: emergency medicine CME, hemophilia, hemophilia A, hemophilia B, von Willebrand disease, vWD, pediatric, children, factor VIII deficiency, factor IX deficiency, von Willebrand factor, vWF, vWF:Ag, vWF:RCo, bleeding disorder, prothrombin time, PT, partial thromboplastin time, PTT, fibrinogen, concentrates, clotting factor, iliopsoas hemorrhage, desmopressin, aminocaproic acid, tranexamic acid, recombinant factor VIIa, rFVIIa