Interim Results of Epidemiological Study of Idiopathic Thrombocytopenic Purpura in Adults in the Russian Federation: Intermediate Results

Abstract

Introduction. Primary immune thrombocytopenia (idiopathic thrombocytopenic purpura, ITP) is a rare autoimmune disorder, whose main clinical manifestation consists in the hemorrhagic syndrome of varying severity. The therapy for ITP is aimed at eliminating hemorrhagic manifestations and maintaining patients’ quality of life. There is no information on the incidence of ITP, as well as on the clinical course and response to the treatment of ITP patients, in the Russian Federation.Aim. To estimate the epidemiological and clinical characteristics of ITP in the adult population of the Russian Federation.Materials and methods. We studied clinical and laboratory data derived from electronic medical records of ITP patients having participated in a multi-centre, prospective, observational, cohort study “Epidemiological and Clinical Characteristics of ITP in Adults in Russia” carried out from 2014 to 2017. The data was processed employing methods of descriptive statistics, as well as frequency analysis, event analysis and analysis of variance. The calculations were performed using the SAS V9.4 statistical package.Results. The incidence of ITP in the adult population in three selected regions of the Russian Federation averaged 2.09 per 100 thousand people per year. The highest incidence rate in gender-age groups was observed in women under 40 years of age mounting to 2.7 per 100 thousand. A gradual increase in the incidence rate from 1.09 to 2.5 per 100 thousand was noted in the men over 60 years of age. A significant correlation was found between the risk of developing the hemorrhagic syndrome, its severity and the platelet count at the onset of the disease (p<0.0001). In 92.2 % of cases, glucocorticosteroid hormones were prescribed as the first line of therapy. The analysis of methods used as the second line of therapy revealed that, although splenectomy is performed relatively frequently, its likelihood has decreased from 26 to 17 % over the past three years. In addition, the proportion of patients being treated with thrombopoietin receptor agonists has increased from 5.9 to 45.7 %.Conclusion: The incidence of ITP in three selected regions of the Russian Federation is comparable to that in other European countries.Conflict of interest: the authors declare no conflict of interest.