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Response to desmopressin of factors XI, X and V in patients with factor VIII deficiency and von Willebrand disease.

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White, Barry Lawler, P Riddell, Anne Nitu-Whalley, Ioana C Hermans, Cédric [UCL] Brown, Simon A

Desmopressin [1-deamino-8-d-arginine vasopressin (DDAVP)] has been successfully used in the treatment of type 1 von Willebrand disease (VWD) and mild haemophilia A (MHA). Data suggest that DDAVP can increase factor XI (FXI) plasma levels and may represent an effective treatment for mild FXI deficiency. We assessed the DDAVP response of FXI coagulant activity (FXI:C), FXI antigen (FXI:Ag), factor V coagulant activity (FV:C), and factor X coagulant activity (FX:C) in 33 individuals with VWD or MHA. DDAVP did not produce a clinically significant increase in FXI:C, FXI:Ag, FX:C or FV:C in any patient. The mean +/- SD FXI:C pre-DDAVP (time 0) and at 1 h post-DDAVP was 90.7 (+/-22.9) U/dl and 92.1 (+/-20.9) U/dl, respectively. The mean (+/-SD) FXI:Ag at time 0 and 1 h was 92.2 (+/-20.1) U/dl and 89.9 (+/-21.3) U/dl, respectively. There was a small reduction at 1 h post-DDAVP in both FV:C, from 101.8 (+/-20.9) U/dl to 97.2 (+/-21.4) U/dl (P < 0.001), and FX:C from 103 (+/-19.5) U/dl to 98.8 (+/-18.7) U/dl (P < 0.001). No significant increase in FXI:C, FXI:Ag, FV:C or FX:C levels was seen at 4 h post-DDAVP. This study failed to demonstrate a clinically significant increase in the levels of FXI, FX or FV following administration of DDAVP.

Document type Article de périodique (Journal article) – Article de recherche
Publication date 2004
Language Anglais
Journal information "British Journal of Haematology" - Vol. 126, no. 1, p. 100-104 (2004)
Peer reviewed yes
Publisher Wiley-Blackwell Publishing Ltd. ((United Kingdom) Oxford)
issn 0007-1048
e-issn 1365-2141
Publication status Publié
Affiliations UCL - MD/MINT - Département de médecine interne
UCL - (SLuc) Service d'hématologie
MESH Subject Adolescent ; Hemophilia A ; Hemostatics ; Humans ; Male ; Middle Aged ; Statistics, Nonparametric ; Treatment Failure ; von Willebrand Diseases ; Adult ; Blood Coagulation Factors ; Child ; Deamino Arginine Vasopressin ; Factor V ; Factor X ; Factor XI ; Female
Links
  1. Lee C. A., Kessler C. M., Varon D., Martinowitz U., Heim M., BOLTON-MAGGS P. H. B., The management of factor XI deficiency, 10.1046/j.1365-2516.1998.440683.x
  2. Bolton-Maggs P.H., Thrombosis and Haemostasis, 67, 314 (1992)
  3. Bolton-Maggs P.H.B, Colvin B.T, Satchi G, Lee C.A, Lucas G.S, Thrombogenic potential of factor XI concentrate, 10.1016/s0140-6736(94)92237-3
  4. Castaman Giancarlo, Ruggeri Marco, Rodeghiero Francesco, Clinical usefulness of desmopressin for prevention of surgical bleeding in patients with symptomatic heterozygous factor XI deficiency, 10.1046/j.1365-2141.1996.d01-1758.x
  5. DiMichele D.M., American Journal of Hematology, 33, 39 (1990)
  6. Federici, Sacco, Stabile, Carpenedo, Zingaro, Mannucci, Optimising local therapy during oral surgery in patients with von Willebrand disease: effective results from a retrospective analysis of 63 cases, 10.1046/j.1365-2516.2000.00370.x
  7. Franchini M., Haematologica, 85, 106 (2000)
  8. Kato A., Cytogenetics and Cell Genetics, 52, 77 (1989)
  9. Kaufmann J. E., Vischer U. M., Cellular mechanisms of the hemostatic effects of desmopressin (DDAVP), 10.1046/j.1538-7836.2003.00190.x
  10. Mannucci P.M., British Journal of Haematology, 30, 81 (1975)
  11. Rao A.K., Thrombosis and Haemostasis, 74, 1071 (1995)
  12. Sadler J.E., Thrombosis and Haemostasis, 71, 520 (1994)
Bibliographic reference White, Barry ; Lawler, P ; Riddell, Anne ; Nitu-Whalley, Ioana C ; Hermans, Cédric ; et. al. Response to desmopressin of factors XI, X and V in patients with factor VIII deficiency and von Willebrand disease.. In: British Journal of Haematology, Vol. 126, no. 1, p. 100-104 (2004)
Permanent URL http://hdl.handle.net/2078/125204