Tags

Type your tag names separated by a space and hit enter

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?
Thromb Res 2009; 123(6):862-8TR

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.

Authors+Show Affiliations

Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, NSW, Australia. emmanuel.favaloro@swahs.health.nsw.gov.auNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Multicenter Study

Language

eng

PubMed ID

19064279

Citation

Favaloro, Emmanuel J., et al. "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, no. 6, 2009, pp. 862-8.
Favaloro EJ, Thom J, Patterson D, et al. 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? Thromb Res. 2009;123(6):862-8.
Favaloro, E. J., Thom, J., Patterson, D., Just, S., Dixon, T., Koutts, J., ... Baker, R. (2009). 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, 123(6), pp. 862-8. doi:10.1016/j.thromres.2008.10.008.
Favaloro EJ, et al. 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. Thromb Res. 2009;123(6):862-8. PubMed PMID: 19064279.
* Article titles in AMA citation format should be in sentence-case
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 -