Type 3 Von Willebrand disease: two clinical cases of a rare disorder

Von Willebrand disease (VWD) is the most common inherited coagulopathy, resulting from a deficiency of von Willebrand factor (VWF), which plays a central role in hemostasis. Type 3 VWD, the rarest and most severe form, is characterized by an almost complete absence of VWF, leading to severe and recurrent bleeding episodes. We report two pediatric cases of third-degree cousins, aged 3 and 2 years, born to consanguineous parents, who presented with severe hemorrhagic syndromes including profuse epistaxis, gingival bleeding, and anemia. Both had markedly prolonged activated partial thromboplastin time, reduced factor VIII, and severely diminished VWF activity and antigen levels, confirming the diagnosis of severe type 3 von Willebrand disease. Management included local hemostasis, avoidance of trauma, and prophylactic administration of plasma-derived VWF/FVIII before invasive procedures. These cases highlight the diagnostic and therapeutic challenges of managing severe inherited bleeding disorders in resource-limited settings, emphasizing the importance of early recognition, family education, and structured follow-up.