OBJECTIVES : To conduct a survey of patients with hereditary coagulopathies and to analyze information on the severity and form of hereditary coagulopathy in comparison with studies performed on similar protocols during the period of the "7 ICD codes" program from 2007 to 2017.

METHODS : Long-term study of the population of Russian patients with various forms of hemophilia was carried out from 2007 to 2017. The questionnaire was sent by mail to 1599 patients with hemophilia and contained questions on clotting factor level and presence of antibodies to it, number of bleeding in last month, number of injections of clotting factors per month, names of used medications, ways of receiving medications, number of ambulance calls and hospitalizations, and the way of administration of medicines. The analysis of epidemiological data was carried out using descriptive statistics methods (mean, median, standard deviation, frequency), the comparative analysis in subgroups was carried out using the statistical χ² and Student’s criteria.

RESULTS : For 10 years, the average age of patients with hemophilia increased from 29.1 to 36.1 years; the proportion of people who did not have bleeding in the last month increased from 11.8 to 21.1%, while the number of episodes of spontaneous hemorrhages in the last month decreased from 3.92 (σ = 1.98, median 5) to 2.59 (σ = 1.63, median 2). The median frequency of administration of IX factor is consistent with the recommendations of the approved Protocol, and VIII factor is close to the recommended. There is practically no viral hepatitis C in the age group up to 25 years, which is associated with the use of blood clotting factors until 2007, almost exclusively in children.

CONCLUSIONS : A number of studies conducted in Russia and abroad have shown that prophylactic treatment in hemophilia compared with "on demand" treatment leads to better clinical outcomes.