Diagnosis and Management of von Willebrand Disease in the United Kingdom

 

 Buy Article Permissions and Reprints

ABSTRACT

The UK treatment strategy for von Willebrand disease (VWD) is based on consensus guidelines produced by the United Kingdom Haemophilia Centre Doctors' Organization (UKHCDO) relating to the diagnosis and management of VWD. Selection of therapeutic products suitable for treatment of this complex inherited bleeding disorder is based on the observed response. Desmopressin (DDAVP), an analog of vasopressin, is the recommended treatment in individuals who respond to this drug on trial infusion. DDAVP clearly has no effect in type 3 VWD but may have variable clinical effect in individuals with other subtypes or may be contraindicated in some cases. In patients where DDAVP treatment is unsuitable, replacement factor concentrate containing von Willebrand factor (VWF) is the recommended alternative. Relevant concentrates are available for all patients in the United Kingdom, and treatment is administered by a network of 67 hemophilia treatment centers that also provide specialist care for individuals diagnosed with VWD. Patients diagnosed with the condition are registered on a national inherited bleeding disorder database administered by the UKHCDO on behalf of the Department of Health to aid in service planning and commissioning. Genetic testing is employed in the United Kingdom in certain situations, which is also performed in accordance with current UKHCDO guidelines.

REFERENCES

• 1 Sadler J E, Budde U, Eikenboom J C Working Party on von Willebrand Disease Classification et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor.  J Thromb Haemost. 2006;  4 (10) 2103-2114
  Google Scholar
• 2 Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease.  Blood. 1987;  69 (2) 454-459
  Google Scholar
• 3 Werner E J, Broxson E H, Tucker E L, Giroux D S, Shults J, Abshire T C. Prevalence of von Willebrand disease in children: a multiethnic study.  J Pediatr. 1993;  123 (6) 893-898
  Google Scholar
• 4 Haemophilia Alliance service specification document. Available at: http://www.ukhcdo.org/docs/HaemAlliance-NatSvsSpec2006.pdf Accessed November 2010
  Google Scholar
• 5 Pasi K J, Collins P W, Keeling D M et al.. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization.  Haemophilia. 2004;  10 (3) 218-231
  Google Scholar
• 6 UK Haemophilia Society .Available at: http://www.haemophilia.org.uk Accessed November 2010
• 7 Office for National Statistics UK population data. Available at: http://www.statistics.gov.uk/cci/nugget.asp?id=6 Accessed October 2010
  Google Scholar
• 8 Sadler J E, Mannucci P M, Berntorp E et al.. Impact, diagnosis and treatment of von Willebrand disease.  Thromb Haemost. 2000;  84 (2) 160-174
  Google Scholar
• 9 Laffan M, Brown S A, Collins P W et al.. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization.  Haemophilia. 2004;  10 (3) 199-217
  Google Scholar
• 10 Keeney S, Bowen D, Cumming A, Enayat S, Goodeve A, Hill M. UK Haemophilia Centre Doctors' Organisation (UKHCDO) . The molecular analysis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organisation Haemophilia Genetics Laboratory Network.  Haemophilia. 2008;  14 (5) 1099-1111
  Google Scholar
• 11 Peake I, Goodeve A. Type 1 von Willebrand disease.  J Thromb Haemost. 2007;  5 (Suppl 1) 7-11
  Google Scholar
• 12 Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. A United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology.  Haemophilia. 2008;  14 (4) 671-684
  Google Scholar
• 13 Hill F G. Haematological disorders.  Clin Obstet Gynaecol. 1982;  9 (1) 75-90
  Google Scholar
• 14 Kadir R A, Sabin C A, Pollard D, Lee C A, Economides D L. Quality of life during menstruation in patients with inherited bleeding disorders.  Haemophilia. 1998;  4 (6) 836-841
  Google Scholar

Steve KeeneyPh.D. 

Deputy Head, Molecular Diagnostics Centre, Manchester Royal Infirmary

Oxford Road, Manchester M13 9WL, UK

Email: steve.keeney@cmft.nhs.uk