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Management of patients with von Willebrand disease: practice of St.Petersburg City Hemophilia Care Centre


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Abstract

Von Willebrand disease (vWD) is a heritable disease, which can be inherited by either autosomal dominant or autosomal recessive, caused by qualitative or quantitative deficiency of von Willebrand factor (vWF) and characterized by excessive bleeding. In Russia 2013 was announced as a year of von Willebrand disease. For elaboration of guidelines on improvement of specific care of children and adults suffering from vWD we carried out a retrospective study. Information regarding the patients registered by regional haematologists with diagnosis of vWD (ICH-10 code D 68.0) was collected. There were examined 86 patients suspected for vWD. The diagnosis was confirmed in 52 (61.6%) patients. This data supported opinion that present status of laboratory service in the Russian regions is not satisfactory for detection of coagulation disorders and final diagnosis justification. Factor VIII concentrate is relatively novel but rare for Russian practice therapeutic approach for bleeding treatment in the patients with vWD. According to the results of the study it was effective in all the cases.


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