Management of von Willebrand disease: A survey on current clinical practice from the haemophilia centres of North America

A. J. Cohen, C. M. Kessler, B. M. Ewenstein, Bruce Ritchie, Margaret Ragni, Michael Tarantino, Leticia Valdez, Bridget Freeman, Georges Rivard, W. Keith Hoots, Edward H. Romond, Patricia McCusker, Linda Shaffer, Joseph Addiego, Thomas Abshire, Man Chiu Poon, Thomas H. Howard, Jeannne M. Lusher, W. Paul Bowman, Richard EdwardsFrederick Rickles, Indira Warrier, Alton L. Lightsey, Parvin Saidi, Eric Larsen, J. Heinreich Joist, David Green, Alan Cohen, Catherine Manno, Donald Mahoney, Cathy Rosenfield, Roshini Kulkarni, Rachelle Nuss, S. R. Seitcher, J. Paul Scott, Brad Lewis, Donna DiMichele, Benjamin Deulbesonic, John Bouhasin, Margaret Heisel, Judy Wilimas, Alberao Pappo, James Harper, Louis Geeraerts, Gerald Gilchrist, Jerry Teitel, D. C. Talbert, Mark Mancino, Felicia Little, Donald Mahoney, Sarah Hawk, Robert Bona, George Buchanan, Jack Lazerson, Barbara A. Konkle, Jerry Powell, Anne Thomas, Martin J. Inwood, Elizabeth Kurczynski, G. C. White, Dennis Gastineau, Eric Werner, David Lilligrap, Robert Janco, Peter Kouides, James L. Harper, Deborah Brown, Carol Kasper, Cindy Lessinger, John D. Bouhasin, Prad Phatak

Research output: Contribution to journalArticle

37 Scopus citations

Abstract

The optimal treatment of patients with von Willebrand's disease (vWD) remains to be defined. Moreover, it has not been firmly established which, if any, commonly measured parameters of von Willebrand factor (vWF) protein in the plasma are useful in guiding treatment. To better understand what guidelines physicians follow in the management of vWD, we surveyed 194 North American physicians who are members of the Hemophilia Research Society. Ninety-nine per cent of responding physicians depend on factor VIII (FVIII):C, vWF:RCo activity and vWF:AG to diagnose vWD, while only 49% use the bleeding time. The minimal goals of treatment for patients undergoing major surgery/trauma or central nervous system haemorrhage were FVIII:C and vWF:RCo activity greater than 80% while levels of more than 50% for minor surgery and dental extractions were considered adequate. Treatment of vWD was based on the type of vWD with type 1 patients being treated most often with desmopressin acetate (DDAVP) alone, types 2A and 2B patients with a combination of DDAVP and a vWF-containing FVIII product, type 3 patients with vWF-containing concentrate. Viral infections, including human immunodeficiency virus, hepatitis A, B and C viruses, and parvovirus have been seen in vWD and the efficacy of viral attenuation processes is a major criterion for the selection of treatment by physicians. Based on this survey, prospective studies need to be designed to address the clinical efficacy, safety and predictive value of laboratory monitoring of patients with vWD.

Original languageEnglish
Pages (from-to)235-241
Number of pages7
JournalHaemophilia
Volume7
Issue number3
DOIs
StatePublished - 5 Jun 2001

Keywords

  • DDAVP
  • Plasma-derived vWF containing factor VIII concentrate
  • Von Willebrand disease

Fingerprint Dive into the research topics of 'Management of von Willebrand disease: A survey on current clinical practice from the haemophilia centres of North America'. Together they form a unique fingerprint.

  • Cite this

    Cohen, A. J., Kessler, C. M., Ewenstein, B. M., Ritchie, B., Ragni, M., Tarantino, M., Valdez, L., Freeman, B., Rivard, G., Hoots, W. K., Romond, E. H., McCusker, P., Shaffer, L., Addiego, J., Abshire, T., Poon, M. C., Howard, T. H., Lusher, J. M., Bowman, W. P., ... Phatak, P. (2001). Management of von Willebrand disease: A survey on current clinical practice from the haemophilia centres of North America. Haemophilia, 7(3), 235-241. https://doi.org/10.1046/j.1365-2516.2001.00498.x