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 Edwards; Frederick 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

doi:10.1046/j.1365-2516.2001.00498.x

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 journal › Article › peer-review

39 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 language	English (US)
Pages (from-to)	235-241
Number of pages	7
Journal	Haemophilia
Volume	7
Issue number	3
DOIs	https://doi.org/10.1046/j.1365-2516.2001.00498.x
State	Published - 2001

Keywords

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

ASJC Scopus subject areas

Hematology
Genetics(clinical)

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10.1046/j.1365-2516.2001.00498.x

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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

Cohen, AJ, Kessler, CM, Ewenstein, BM, Ritchie, B, Ragni, M, Tarantino, M, Valdez, L, Freeman, B, Rivard, G, Hoots, WK, Romond, EH, McCusker, P, Shaffer, L, Addiego, J, Abshire, T, Poon, MC, Howard, TH, Lusher, JM, Bowman, WP, Edwards, R, Rickles, F, Warrier, I, Lightsey, AL, Saidi, P, Larsen, E, Joist, JH, Green, D, Cohen, A, Manno, C, Mahoney, D, Rosenfield, C, Kulkarni, R, Nuss, R, Seitcher, SR, Scott, JP, Lewis, B, DiMichele, D, Deulbesonic, B, Bouhasin, J, Heisel, M, Wilimas, J, Pappo, A, Harper, J, Geeraerts, L, Gilchrist, G, Teitel, J, Talbert, DC, Mancino, M, Little, F, Mahoney, D, Hawk, S, Bona, R, Buchanan, G, Lazerson, J, Konkle, BA, Powell, J, Thomas, A, Inwood, MJ, Kurczynski, E, White, GC, Gastineau, D, Werner, E, Lilligrap, D, Janco, R, Kouides, P, Harper, JL, Brown, D, Kasper, C, Lessinger, C, Bouhasin, JD & Phatak, P 2001, 'Management of von Willebrand disease: A survey on current clinical practice from the haemophilia centres of North America', Haemophilia, vol. 7, no. 3, pp. 235-241. https://doi.org/10.1046/j.1365-2516.2001.00498.x

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

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.",

keywords = "DDAVP, Plasma-derived vWF containing factor VIII concentrate, Von Willebrand disease",

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

year = "2001",

doi = "10.1046/j.1365-2516.2001.00498.x",

language = "English (US)",

volume = "7",

pages = "235--241",

journal = "Haemophilia",

issn = "1351-8216",

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T1 - Management of von Willebrand disease

T2 - A survey on current clinical practice from the haemophilia centres of North America

AU - Cohen, A. J.

AU - Kessler, C. M.

AU - Ewenstein, B. M.

AU - Ritchie, Bruce

AU - Ragni, Margaret

AU - Tarantino, Michael

AU - Valdez, Leticia

AU - Freeman, Bridget

AU - Rivard, Georges

AU - Hoots, W. Keith

AU - Romond, Edward H.

AU - McCusker, Patricia

AU - Shaffer, Linda

AU - Addiego, Joseph

AU - Abshire, Thomas

AU - Poon, Man Chiu

AU - Howard, Thomas H.

AU - Lusher, Jeannne M.

AU - Bowman, W. Paul

AU - Edwards, Richard

AU - Rickles, Frederick

AU - Warrier, Indira

AU - Lightsey, Alton L.

AU - Saidi, Parvin

AU - Larsen, Eric

AU - Joist, J. Heinreich

AU - Green, David

AU - Cohen, Alan

AU - Manno, Catherine

AU - Mahoney, Donald

AU - Rosenfield, Cathy

AU - Kulkarni, Roshini

AU - Nuss, Rachelle

AU - Seitcher, S. R.

AU - Scott, J. Paul

AU - Lewis, Brad

AU - DiMichele, Donna

AU - Deulbesonic, Benjamin

AU - Bouhasin, John

AU - Heisel, Margaret

AU - Wilimas, Judy

AU - Pappo, Alberao

AU - Harper, James

AU - Geeraerts, Louis

AU - Gilchrist, Gerald

AU - Teitel, Jerry

AU - Talbert, D. C.

AU - Mancino, Mark

AU - Little, Felicia

AU - Mahoney, Donald

AU - Hawk, Sarah

AU - Bona, Robert

AU - Buchanan, George

AU - Lazerson, Jack

AU - Konkle, Barbara A.

AU - Powell, Jerry

AU - Thomas, Anne

AU - Inwood, Martin J.

AU - Kurczynski, Elizabeth

AU - White, G. C.

AU - Gastineau, Dennis

AU - Werner, Eric

AU - Lilligrap, David

AU - Janco, Robert

AU - Kouides, Peter

AU - Harper, James L.

AU - Brown, Deborah

AU - Kasper, Carol

AU - Lessinger, Cindy

AU - Bouhasin, John D.

AU - Phatak, Prad

PY - 2001

Y1 - 2001

N2 - 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.

AB - 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.

KW - DDAVP

KW - Plasma-derived vWF containing factor VIII concentrate

KW - Von Willebrand disease

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

Abstract

Keywords

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