title: Diagnosis and management of adult patients with von Willebrand disease in South Australia.
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.
authors.affiliation: Haematology Division, Institute of Medical and Veterinary Science, Adelaide, South Australia. susan.rodgers@health.sa.gov.au
authors.forename: Susan Elizabeth
authors.initials: SE
authors.lastname: Rodgers
authors.forename: John Viner
authors.initials: JV
authors.lastname: Lloyd
authors.forename: Hilda Magdalena
authors.initials: HM
authors.lastname: Mangos
authors.forename: Elizabeth Mary
authors.initials: EM
authors.lastname: Duncan
authors.forename: Simon J
authors.initials: SJ
authors.lastname: McRae
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D016208:Databases, Factual; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005260:Female; D005820:Genetic Testing; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D013021:South Australia; D014148:Tranexamic Acid / Q000627:therapeutic use; D055815:Young Adult; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D014841:von Willebrand Factor / Q000235:genetics
chemical_list: D000933:Antifibrinolytic Agents; D006490:Hemostatics; D014841:von Willebrand Factor; D014148:Tranexamic Acid; D003894:Deamino Arginine Vasopressin