Abstract

Background:Primary immune thrombocytopenia (idiopathic thrombocytopenic purpura ‐ ITP) is a rare autoimmune disease, the main clinical manifestations of which are hemorrhagic syndrome of varying severity. The incidence of ITP in the adult population of different countries varies from 1.6 to 12.5/100,000 person‐years. ITP register started in the Russian Federation (RF) In December 2014. This is multicenter, prospective observational cohort study “Epidemiological and clinical characteristics of ITP in adults in Russia”.Aims:Assessment of the epidemiological characteristics of ITP in the adult population of the RF.Methods:Clinical and laboratory data of the register “Epidemiological and clinical characteristics of ITP in adults in Russia”. Methods of descriptive statistics, frequency, variance analysis and event analysis used in statistical processing.Results:The incidence of ITP in the adult population of the Russian Federation averaged 2.09/100,000 people. The highest morbidity of 2.7/100,000 recorded among women under 40 years of age. While in men there was a gradual increase in the incidence from 1.09 to 2.5/100,000 people over the age of 60 years. The result obtained is comparable with the data of the registers of other European countries. An analysis of incidence in the sex‐age strata showed that men have a tendency to an increase in the incidence of ITP from 1.09 at the age of 40, to 2.98 at the age of 60. Similar changes in the incidence in women of different age groups not detected. It is noteworthy that in the age group over 60 years, the incidence in men and women is almost the same, whereas data from epidemiological studies in other European countries showed a significant predominance in this group of men.There was a significant relationship between the risk of development, the degree of bleeding and the number of platelets in the onset of the disease: the severity of hemorrhagic syndrome increases with the number of platelets below 50x109/l (p < 0.0001). In 92.2% of cases glucocorticosteroid hormones prescribed as the first line of therapy. Analysis of methods of treatment used as a second line therapy demonstrated that the probability of splenectomy the last 3 years decreased from 26 to 17%, while the number of patients receiving thrombopoetin receptors agonists increased from 5.9 to 45.7%.Summary/Conclusion:The epidemiological data are comparable with those in other European countries. Geographical variations of incidence in different regions of the Russian Federation require further study.

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