TY - JOUR T1 - 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? AU - Favaloro,Emmanuel J, AU - Thom,Jim, AU - Patterson,David, AU - Just,Sarah, AU - Dixon,Tracy, AU - Koutts,Jerry, AU - Baccala,Maria, AU - Rowell,John, AU - Baker,Ross, Y1 - 2008/12/07/ PY - 2008/07/05/received PY - 2008/09/16/revised PY - 2008/10/14/accepted PY - 2008/12/10/pubmed PY - 2009/7/18/medline PY - 2008/12/10/entrez SP - 862 EP - 8 JF - Thrombosis research JO - Thromb Res VL - 123 IS - 6 N2 - 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. SN - 0049-3848 UR - https://www.unboundmedicine.com/medline/citation/19064279/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 L2 - https://linkinghub.elsevier.com/retrieve/pii/S0049-3848(08)00494-5 DB - PRIME DP - Unbound Medicine ER -