TY - JOUR
T1 - Treatment of acquired von Willebrand syndrome in childhood
AU - Callaghan, Michael U.
AU - Wong, Trisha E.
AU - Federici, Augusto B.
PY - 2013/9/19
Y1 - 2013/9/19
N2 - 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?
AB - 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?
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U2 - 10.1182/blood-2012-10-435719
DO - 10.1182/blood-2012-10-435719
M3 - Review article
C2 - 23878141
AN - SCOPUS:84887594608
SN - 0006-4971
VL - 122
SP - 2019
EP - 2022
JO - Blood
JF - Blood
IS - 12
ER -