We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Acquired von Willebrand Syndrome in Childhood and Adolescence.
- Authors
Will, Andrew M.
- Abstract
Acquired von Willebrand syndrome (AVWS) is a rare condition usually seen in middle-aged and elderly patients. This review summarizes the clinical features, etiology, pathophysiology, classification, and management of 113 children and adolescents with AVWS. There were nine underlying conditions associated with the occurrence of AVWS in younger patients: patients taking valproic acid, congenital heart disease, renal tumors, glycogen storage disease type 1a, hypothyroidism, systemic lupus erythematosus, E/beta0 thalassemia, acute lymphoblastic leukemia, and one case following an Epstein-Barr infection. Diagnostic criteria included recent onset bleeding or bruising in a patient with no previous personal history of bleeding or family history of a bleeding disorder with laboratory tests suggestive of von Willebrand disease (VWD) or the presence of abnormal von Willebrand multimers. The pathophysiology and management of acute bleeding was dependent on the primary underlying disease. Successful treatment of the underlying disease was almost always associated with sustained correction of the AVWS.
- Subjects
VON Willebrand disease; VON Willebrand factor; BLOOD coagulation disorders; CONGENITAL heart disease; VALPROIC acid; KIDNEY tumors; GLYCOGEN storage disease; THALASSEMIA; EPSTEIN-Barr virus diseases
- Publication
Journal of Coagulation Disorders, 2010, Vol 2, Issue 2, p1
- ISSN
2041-7969
- Publication type
Article