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Low von Willebrand factor in pediatric patients: Retrospective analysis of 293 cases informs diagnostic and therapeutic decision making
Author(s) -
Shui Michelle,
D'Angelo Loren,
Croteau Stacy E.
Publication year - 2020
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.28497
Subject(s) - medicine , von willebrand factor , bleed , retrospective cohort study , intensive care medicine , surgery , platelet
Low von Willebrand factor (VWF) poses diagnostic and therapeutic challenges for predicting bleed risk and need for empiric hemostatic therapy, particularly in children. Retrospective review identified 293 low VWF pediatric patients and investigated clinical and laboratory features. Low activity to antigen ratio was observed in 142 patients. When evaluation included VWF activity with gain‐of‐function glycoprotein Ib fragments (VWF:GPIbM) assay or VWF exon 28 sequencing, low VW was excluded in the majority (62%) of these patients. Application of a condensed bleeding assessment tool to quantify bleeding symptoms and use of the VWF:GPIbM assay may reduce the number of patients with bleed risk anxiety and unnecessary hemostatic management plans.