Treatment of acquired von Willebrand syndrome in childhood

Michael U. Callaghan, Trisha E. Wong, Augusto B. Federici

Research output: Contribution to journalReview article

13 Scopus citations

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 amass 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?

Original languageEnglish (US)
Pages (from-to)2019-2022
Number of pages4
JournalBlood
Volume122
Issue number12
DOIs
StatePublished - Sep 19 2013

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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