Pediatric vascular hemophilia

Vascular hemophilia (von Willebrand disease, vWD) is a complex bleeding disorder with defective hemostatic function. This disease was first reported by Erik von Willebrand in 1926 and was thought to be caused by platelet dysfunction or vascular abnormalities. Pediatric patients are often caused by familial inheritance, which is a more common type of hereditary bleeding disorders that can be affected by both men and women. It is now clear that abnormalities in the quality or quantity of von Willebrand Factor (vWF) cause vWD with a reduction in factor Ⅶ procoagulant activity (Ⅷ: C). Clinically, skin and mucous membranes have varying degrees of bleeding tendency, platelet adhesion is reduced, and bleeding time is prolonged. Acquired vascular hemophilia can occur on the basis of a variety of diseases, and a few patients can be free of underlying diseases. Rarely in children.