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patient fviii willebrand ddavp von willebrand disease vwd type 1 disease mutation response factor level blood vwf multimers vwf parameters study multimer group type 1 type 2 von willebrand factor table >250 erasmus univ rotterdam fviii /vwf ratio treatment jak 2v mutation michiel surgery value parameter vwf /fviii loading dose recessive marrow haemate-p diagnosis jak 2v type 2 vwd thromb haematol loading vwd patients criteria missense bone marrow features presence platelet domain thrombocythemia infusion blood group /fviii alphanate allele absence prophylaxi hemate-p rotterdam vwf deficiency european half life times vwd type 3 erasmu abo blood group deficiency plasma berneman gadisseur pattern schroyen effect recovery megakaryocyte thrombosis downloaded cat.sagepub.com type 3 vwd dosing defect