Journal article
Desmopressin therapy to assist the functional identification and characterisation of von Willebrand disease: Differential utility from combining two (VWF:CB and VWF:RCo) von Willebrand factor activity assays?
Thrombosis Research, Vol.123(6), pp.862-868
2009
Abstract
We performed a retrospective audit of desmopressin (DDAVP) usage to assist in the functional characterisation of von Willebrand disease (VWD). Data was evaluated for 208 patients, comprising those with VWD (Type 1 [n = 160], Type 2A [n = 19], Type 2M [n = 10]), plus 19 individuals with haemophilia or carriers of haemophilia. Laboratory testing comprised pre- and post-DDAVP evaluation of factor VIII (FVIII:C), von Willebrand factor (VWF) antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo) activity, VWF collagen binding (VWF:CB) activity, and in one laboratory an alternate VWF activity assay. In brief, combined usage of VWF:RCo and VWF:CB appears to provide improved functional characterisation and/or ‘classification’ of VWD types, in particular better differentiation of Type 2A and 2M VWD, and clearer validation of a Type 1 VWD diagnosis. Thus, (i) Type 1 VWD displayed generally good absolute and relative rises in all test parameters, although relative rises were greatest for FVIII:C and VWF:CB, and CB/Ag ratio increases overshadowed those for RCo/Ag; (ii) Type 2A VWD patients showed good absolute and relative rises in both FVIII:C and VWF:Ag, but poor absolute rises in both VWF:CB and VWF:RCo; although small rises in both CB/Ag and RCo/Ag were also observed, both ratios tended to remain below 0.7; (iii) finally, Type 2 M VWD patients generally showed good absolute and relative rises in FVIII:C, VWF:Ag and VWF:CB, but a poor absolute and relative rise in VWF:RCo; thus, there were good rises in CB/Ag ratios but little change in RCo/Ag, which tended to remain below 0.7. Future multi-centre prospective investigations are warranted to validate these findings and to investigate their therapeutic implications.
Details
- Title
- Desmopressin therapy to assist the functional identification and characterisation of von Willebrand disease: Differential utility from combining two (VWF:CB and VWF:RCo) von Willebrand factor activity assays?
- Authors/Creators
- E.J. Favaloro (Author/Creator) - Westmead HospitalJ. Thom (Author/Creator) - Royal Perth HospitalD. Patterson (Author/Creator) - Canterbury Health LaboratoriesS. Just (Author/Creator) - Department of Haematology, Pathology Queensland, Royal Brisbane Hospital, Qld, AustraliaT. Dixon (Author/Creator) - Fremantle HospitalJ. Koutts (Author/Creator) - Westmead HospitalM. Baccala (Author/Creator) - Royal Perth HospitalJ. Rowell (Author/Creator) - Department of Haematology, Pathology Queensland, Royal Brisbane Hospital, Qld, AustraliaR. Baker (Author/Creator) - Royal Perth Hospital
- Publication Details
- Thrombosis Research, Vol.123(6), pp.862-868
- Identifiers
- 991005542076307891
- Murdoch Affiliation
- Institute for Immunology and Infectious Diseases
- Language
- English
- Resource Type
- Journal article
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