Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis · 2019
Recombinant Von Willebrand factor concentrate in 2A Von Willebrand disease: comparison to plasma-derived Von Willebrand factor concentrate therapy.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Rajpurkar Madhvi, Frey Mary Jane, Sabo Cynthia, Hollon Wendy
Аннотация
: 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.