Treatment

The treatment of vWD depends on the type of disease that is present and the severity of bleeding. IĀ§ble...212-11 shows the recommended therapy for patients with vWD. Desmopressin is the mainstay of therapy for patients with type I vWD; plasma products that contain vWF are used for types II and III. Desmopressin can be administered subcutaneously or intravenously at a dose of 0.3 Mg/kg of body weight. This dose is given every 12 h to a total of three or four doses before temporary tachyphylaxis develops. A concentrated form of desmopressin can be administered intranasally. The dose is 150 Mg (one metered dose) for patients weighing less than 50 kg and 300 Mg for patients weighing 50 kg, more. In a setting where treatment is essential and desmopressin or the specifically recommended factor VIII concentrates are not available, cryoprecipitate can be used to replace the vWF that is needed for hemostasis. Each bag of cryoprecipitate contains about 100 units of vWF activity. Cryoprecipitate should only be used in emergencies because there is a small risk of viral transmission. Women with vWD are often treated with hormonal agents (estrogens and/or progesterones), which cause an increase in the vWF activity. Bleeding associated with dental procedures can often be managed with fibrinolytic inhibitor agents such as EACA and tranexamic acid.

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