Management of patients with von Willebrand disease: practice of St Petersburg City Hemophilia Care Centre
https://doi.org/10.18821/0234-5730-2016-61-1-49-53
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.
Keywords
on Willebrand disease,
von Willebrand factor,
desmopressin,
von Willebrand factor antigen,
ristocetin cofactor activity
Supporting agencies: Biological samples delivery to St Petersburg was supported by the pharmaceutical company “GlaxoSmithKline” (United Kingdom). All the other study activities had no sponsorship.
About the Authors
T. A. Andreeva
City Hemophilia Care Centre
Russian Federation
Russian Federation
Andreeva Tatiana А., MD, PhD, head of City Hemophilia Care Centre, City Outpatient Medical Clinic No37
191186, St. Petersburg
I. A. Lavrichenko
City Hemophilia Care Centre
Russian Federation
Russian Federation
191186, St. Petersburg
N. I. Klimova
City Hemophilia Care Centre
Russian Federation
191186, St. Petersburg
Russian Federation
191186, St. Petersburg
O. E. Zalepukhina
City Hemophilia Care Centre
Russian Federation
191186, St. Petersburg
Russian Federation
191186, St. Petersburg
V. N. Konstantinova
City Hemophilia Care Centre
Russian Federation
191186, St. Petersburg
Russian Federation
191186, St. Petersburg
A. V. Kim
City Hemophilia Care Centre
Russian Federation
191186, St. Petersburg
Russian Federation
191186, St. Petersburg
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For citations:
Andreeva T.A., Lavrichenko I.A., Klimova N.I., Zalepukhina O.E., Konstantinova V.N., Kim A.V. Management of patients with von Willebrand disease: practice of St Petersburg City Hemophilia Care Centre. Russian journal of hematology and transfusiology. 2016;61(1):49-53. (In Russ.) https://doi.org/10.18821/0234-5730-2016-61-1-49-53
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