Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder.

摘要: von Willebrand disease (VWD) is the most common inherited bleeding disorder and arises from deficiencies and/or defects in plasma protein factor (VWF). The current report overviews diagnosis management of VWD as reflected by differential processes applied within centers around world. prevalence VWD, well frequency different types, also reported. data varies according to methodology used, with epidemiological/population screening estimates approximating 1% population (or 10,000 cases per million population), several orders magnitude higher than disorders registry or regional/center analysis (which instead range 50% all cases, does specific qualitative types (i.e., 2A, 2B, 2M, 2N). Although type 2A considered form 2 some reports workers consider 2M be as, more, common. not a "true" given its platelet origin, platelet-type only rarely identified. Finally, differs geographic region. Most developed countries use standard therapy, employing desmopressin (DDAVP) wherever possible, concentrate other situations, antifibrinolytic therapy required. In contrast, relative high cost unavailability concentrates developing countries, sometimes DDAVP, requires strategies applied.