Recombinant von Willebrand factor concentrate in 2A von Willebrand disease: Comparison to plasma-derived von Willebrand factor concentrate therapy

Madhvi Rajpurkar, Mary Jane Frey, Cynthia Sabo, Wendy Hollon

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Type 2A sub-type of Von Willebrand disease (VWD) is characterized by the loss of high molecular weight multimers. Several plasma-derived Von Willebrand factor concentrates (PD-VWFC) are available for treatment and recently a recombinant VWF concentrate (rVWFC) has been approved for use in VWD for adults in the United States. We describe a patient with Type 2A VWD who had persistent refractory epistaxis despite treatment with PD-VWFC. We describe differences in VWF multimeric composition and Factor VIII (FVIII) levels after plasma-derived and rVWF concentrates. Despite similar VWF levels, VWF multimeric composition after PD-VWFC remained abnormal while it corrected with rVWFC. Post-PD-VWFC, high levels of FVIII were seen, which were not observed after rVWFC. Recombinant VWFC may offer some advantages over PD-VWFC. This finding needs to be confirmed in larger studies.

Original languageEnglish
Pages (from-to)168-170
Number of pages3
JournalBlood Coagulation and Fibrinolysis
Volume30
Issue number4
DOIs
StatePublished - Jun 1 2019

Keywords

  • Von Willebrand disease
  • plasma derived Von Willebrand factor concentrate
  • recombinant Von Willebrand factor concentrate

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