Management of von Willebrand disease in developing countries

A variety of treatment options are available at present for patients with von Willebrand disease (vWD), some of which are affordable for patients in developing countries. For most patients who have type 1 vWD, desmopressin acetate (DDAVP) is the treatment of first choice, at least for minor bleedings and for prophylaxis. It is advisable, however, to use a test dose first to note the patient's response. DDAVP is safe to use, affordable, and easy to administer. However, most patients with type 2 vWD and all patients with type 3 fail to respond to DDAVP. For these patients, other options are used. Almost all patients with vWD will benefit from fresh frozen plasma and from cryoprecipitate, and these are viable options for developing countries. Both have certain disadvantages, but they can, depending upon the circumstances and facilities, be produced in developing countries. In developed countries, factor VIII/von Willebrand factor concentrates are widely used, especially for major bleedings and for surgeries on these patients. These concentrates are safe and virus inactivated, but costly. Ancillary treatment modalities such as antifibrinolytic agents and certain hormones are usually given in conjunction with other modalities. The treatment of patients with antibodies to vWF is also described, and monitoring needs during therapy are reviewed.