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

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., Edwards, R., Rickles, F., Warrier, I., Lightsey Jr. A. L., Saidi, P., Larsen, E., Joist, J. H., Green, D., Cohen, A., Manno, C., Mahoney, D., Rosenfield, C., Kulkarni, R., Nuss, R., Seitcher, S. R., Scott, J. P., Lewis, B., DiMichele, D., Deulbesonic, B., Bouhasin, J., Heisel, M., Wilimas, J., Pappo, A., Harper, J., Geeraerts, L., Gilchrist, G., Teitel, J., Talbert, D. C., Mancino, M., Little, F., Mahoney Jr., D., Hawk, S., Bona, R., Buchanan, G., Lazerson, J., Konkle, B. A., Powell, J., Thomas, A., Inwood, M. J., Kurczynski, E., White, G. C., Gastineau, D., Werner, E., Lilligrap, D., Janco, R., Kouides, P., Harper, J. L., Brown, D., Kasper, C., Lessinger, C., Bouhasin, J. D. and 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. doi:10.1046/j.1365-2516.2001.00498.x

Author 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.
Edwards, R.
Rickles, F.
Warrier, I.
Lightsey Jr. A. L.
Saidi, P.
Larsen, E.
Joist, J. H.
Green, D.
Cohen, A.
Manno, C.
Mahoney, D.
Rosenfield, C.
Kulkarni, R.
Nuss, R.
Seitcher, S. R.
Scott, J. P.
Lewis, B.
DiMichele, D.
Deulbesonic, B.
Bouhasin, J.
Heisel, M.
Wilimas, J.
Pappo, A.
Harper, J.
Geeraerts, L.
Gilchrist, G.
Teitel, J.
Talbert, D. C.
Mancino, M.
Little, F.
Mahoney Jr., D.
Hawk, S.
Bona, R.
Buchanan, G.
Lazerson, J.
Konkle, B. A.
Powell, J.
Thomas, A.
Inwood, M. J.
Kurczynski, E.
White, G. C.
Gastineau, D.
Werner, E.
Lilligrap, D.
Janco, R.
Kouides, P.
Harper, J. L.
Brown, D.
Kasper, C.
Lessinger, C.
Bouhasin, J. D.
Phatak, P.
Title Management of von Willebrand disease: a survey on current clinical practice from the haemophilia centres of North America
Journal name Haemophilia   Check publisher's open access policy
ISSN 1351-8216
Publication date 2001-05-01
Year available 2001
Sub-type Article (original research)
DOI 10.1046/j.1365-2516.2001.00498.x
Open Access Status Not yet assessed
Volume 7
Issue 3
Start page 235
End page 241
Total pages 7
Place of publication Chichester, West Sussex, United Kingdom
Publisher Wiley-Blackwell Publishing
Language eng
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.
Keyword DDAVP
Plasma-derived vWF containing factor VIII concentrate
Von Willebrand disease
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 30 times in Thomson Reuters Web of Science Article | Citations
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