Acquired von Willebrand Syndrome in Childhood and Adolescence

Review article

Acquired von Willebrand Syndrome in Childhood and Adolescence

M Andrew Will

Affiliation: Royal Manchester Children’s Hospital, Manchester, UK

Submission date: 2nd July 2009, Revision date: 29th August 2009, Acceptance date: 5th September 2009


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.

Keywords: acquired von Willebrand syndrome, children, adolescents, valproate, congenital heart disease, Wilms, hypothyroidism, systemic lupus erythematosus
Correspondence: Ward 84, Royal Manchester Children’s Hospital, Oxford Road, Manchester M13 9WL, UK