| Literature DB >> 23878141 |
Michael U Callaghan1, Trisha E Wong, Augusto B Federici.
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?Entities:
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Year: 2013 PMID: 23878141 DOI: 10.1182/blood-2012-10-435719
Source DB: PubMed Journal: Blood ISSN: 0006-4971 Impact factor: 22.113