title: Treatment of acquired von Willebrand syndrome in childhood.
abstract: A 3-1/2-year-old male with no personal or family history of bleeding disorders presented with abdominal distension, epistaxis, and anemia (hemoglobin 8.2 g/dL). A magnetic resonance imaging scan of the abdomen demonstrated a mass arising from the left kidney. Preoperative laboratory studies revealed a prolonged activated partial thromboplastin time of 49.2 seconds, a normal prothrombin time of 12.4 seconds, and a platelet count of 230, 000/μL. Further testing revealed factor VIII (FVIII) activity of 16%, factor IX (FIX) activity of 74%, von Willebrand factor (VWF) activity of 12%, VWF antigen activity of 31%, and decreased high-molecular-weight VWF multimers consistent with acquired von Willebrand syndrome (AVWS). What is the best treatment for this child?
authors.affiliation: Wayne State University and Children's Hospital of Michigan, Detroit, MI;
authors.forename: Michael U
authors.initials: MU
authors.lastname: Callaghan
authors.forename: Trisha E
authors.initials: TE
authors.lastname: Wong
authors.forename: Augusto B
authors.initials: AB
authors.lastname: Federici
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D002318:Cardiovascular Diseases / Q000150:complications; D002648:Child; D002675:Child, Preschool; D064420:Drug-Related Side Effects and Adverse Reactions; D006801:Humans; D007037:Hypothyroidism / Q000150:complications / Q000378:metabolism; D009369:Neoplasms / Q000150:complications / Q000378:metabolism; D014842:von Willebrand Diseases / Q000209:etiology* / Q000628:therapy*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D001323:Autoantibodies; D014841:von Willebrand Factor