А.V. КОSTERINA

Kazan State Medical University of the MH of RF, 49 Butlerov Str., Kazan, Russian Federation, 420012

  Коsterina A.V. ― Assistant of the Department of Hospital Therapy, e-mail: avakost@mail.ru

Pathophysiology and pathogenesis of clinical manifestations of von Willebrand disease are given. Classification of vWD based on qualitative and quantitative changes of von Willebrand factor (vWF) is given, the causes of secondary von Willebrand disease are explained. The prognosis of the disease and the existing scales of assessment and self-assessment of bleeding in case of this disease are presented. Tests used to make a diagnose and differentiate subtypes of von Willebrand disease are described. Differential diagnostics of von Willebrand disease is considered. Recommended methods of treatment are presented depending on the subtype of the disease, possible errors and complications in the therapy. Data on the treatment of von Willebrand disease during pregnancy are submitted.

Key words: von Willebrand disease, von Willebrand factor, procoagulant factor VIII, ristocetin cofactor activity, desmopressin (DDAVP), recombinant von Willebrand factor (rVWF), concentrates containing von Willebrand factor (VWF) and factor VIII (FVIII).

(For citation: Коsterina A.V. Issues of diagnosis and treatment of von Willebrand disease (vWD) . Practical Medicine. 2018)

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