von Willebrand's disease: a report from a meeting in the Åland islands.
von Willebrand's disease (VWD) is probably the most common bleeding disorder, with some studies indicating that up to 1% of the population may have the condition. Over recent years interest in VWD has fallen compared to that of haemophilia, partly the result of focus on blood-borne diseases such as HIV and hepatitis. Now the time has come to revisit VWD, and in view of this some 60 international physicians with clinical and scientific interest in VWD met over 4 days in 2010 in the Åland islands to discuss state-of-the-art issues in the disease. The Åland islands are where Erik von Willebrand had first observed a bleeding disorder in a number of members of a family from Föglö, and 2010 was also the 140th anniversary of his birth. This report summarizes the main papers presented at the symposium; topics ranged from genetics and biochemistry through to classification of VWD, pharmacokinetics and laboratory assays used in the diagnosis of the disease, inhibitors, treatment guidelines in different age groups including the elderly who often have comorbid conditions that present challenges, and prophylaxis. Other topics included managing surgeries in patients with VWD and the role of FVIII in VWF replacement, a controversial subject.
Lund University, Department of Hematology and Coagulation Disorders Skåne University Hospital, Malmö, Sweden.
E
E
Berntorp
I
I
Peake
U
U
Budde
M
M
Laffan
R
R
Montgomery
J
J
Windyga
A
A
Goodeve
P
P
Petrini
M
M
von Depka
W
W
Miesbach
D
D
Lillicrap
A B
AB
Federici
R
R
Lassila
G
G
White
D005169:Factor VIII / Q000493:pharmacokinetics / Q000627:therapeutic use*; D015316:Genetic Therapy; D006801:Humans; D017410:Practice Guidelines as Topic; D012537:Scandinavian and Nordic Countries; D006113:United Kingdom; D014481:United States; D014842:von Willebrand Diseases / Q000145:classification / Q000378:metabolism / Q000628:therapy*; D014841:von Willebrand Factor / Q000378:metabolism / Q000493:pharmacokinetics / Q000627:therapeutic use*
D014841:von Willebrand Factor; D005169:Factor VIII