Bleeding manifestations in males with von Willebrand disease

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia 2004-03, Vol.10 (2), p.162-168
• Main Authors: Ziv, O, Ragni, M. V.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Blood Coagulation Tests; Child; Child, Preschool; Factor VIII - analysis; Hematoma - etiology; Hemorrhage - etiology; Humans; Infant; Male; males with bleeding disorders; Middle Aged; postoperative bleeding; Postoperative Hemorrhage - etiology; Preoperative Care; preoperative screening; Retrospective Studies; von Willebrand disease; von Willebrand Diseases - blood; von Willebrand Diseases - complications; von Willebrand Factor - analysis
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1111/j.1365-2516.2003.00871.x

von Willebrand disease (vWD) is the most common congenital bleeding disorder in the USA, affecting 1–3% of the population. Previously characterizing the bleeding symptoms in females with type 1 vWD, we evaluated 42 males with type 1 vWD, mean age 16 years (1–64), of whom 24 (57%) presented with bleeding symptoms. The most common initial symptom was postoperative bleeding (26%). The most common bleeding symptoms ever were epistaxis (53%), bruising (50%), postoperative bleeding (47%), haematomas (29%) and oral bleeding (29%). Of postoperative bleeding, ear/nose/throat (44%), dental (17%) and circumcision bleeding (22%) occurred at a median 10 years of age, despite a previous bleeding or family history in 89%. Complications included anaemia in five (12%), neurological sequelae after subdural haematoma and tonsillectomy in two (5%), transfusion‐associated hepatitis C in two (5%) and degenerative joint disease after traumatic haemarthroses in one (2%). The bleeding time (BT) was prolonged in 83%, and the ristocetin cofactor (vW:RCoF) and factor VIII (FVIII:C) decreased in 64% and 43%, respectively. Haemarthroses and haematoma formation were associated with a longer activated partial thromboplastin time (APTT) (P