Semin Thromb Hemost 2011; 37(5): 535-541
DOI: 10.1055/s-0031-1281040
© Thieme Medical Publishers

Diagnosis and Management of Adult Patients with von Willebrand Disease in South Australia

Susan Elizabeth Rodgers1 , John Viner Lloyd1 , Hilda Magdalena Mangos2 , Elizabeth Mary Duncan1 , Simon J. McRae1
  • 1Haematology Division, Institute of Medical and Veterinary Science, Adelaide, South Australia
  • 2Department of Haematology, Dunedin Hospital, Private bag 1921, Dunedin 9054, New Zealand
Further Information

Publication History

Publication Date:
18 November 2011 (online)

ABSTRACT

We have analyzed the databases for von Willebrand disease (VWD) from the hemophilia center for adult patients with bleeding disorders in South Australia. We define the prevalence of types of VWD to determine the proportion of who would be treated with factor (F) VIII/von Willebrand factor (VWF) concentrate to prevent or control hemorrhage. In severe or moderately severe patients, we use plasma-derived FVIII/VWF concentrate, and for mild to severe cases, we use desmopressin plus tranexamic acid. There are 103 patients with VWF ristocetin (RCo) ≤50 IU/dL: 38 (37%) severe (VWF:RCo <10 IU/dL), 28 (27%) moderate (VWF:RCo 10 to 29 IU/dL), and 37 (36%) mild (VWF:RCo 30 to 50 IU/dL). Hence in 66 (64%), FVIII/VWF concentrate is the mainstay of treatment. The prevalence of VWD in our region according to data from our center is ~1 per 12,000. A total of 52% of patients are type 1, 44% type 2, and 5% type 3. In our experience, type 2M (45% of type 2) is much more common than types 2A and 2B (each 9% of type 2). Mutation detection is useful for identifying some subtypes of VWD.

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Susan Elizabeth RodgersM.App.Sc. 

Senior Medical Scientist, Division of Haematology, Institute of Medical and Veterinary Science

P.O. Box 14 Rundle Mall, Adelaide, South Australia 5000

Email: susan.rodgers@health.sa.gov.au