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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.

Introduction. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia. Splenectomy is one the most effective treatment methods for this disorder.Aim — to evaluate the effi cacy and safety of a splenectomy in patients with primary immune thrombocytopenia.Subjects and methods. 111 patients (31 males, 80 females) with primary immune thrombocytopenia who were hospitalized to perform laparoscopic splenectomy were included in a prospective study conducted at the National Research Center for Hematology from 2015 to 2019. Disease duration from onset to splenectomy was from 1 month to 51 years. Response to the splenectomy, complications, and correlation with immediate preoperative platelet count were analyzed.Results. Complete response was achieved in 79 (71.2 %) cases, a partial response was achieved in 11 (9.9 %) cases, and in 21 (18.9 %) cases there was no response. Immediate preoperative platelet count was signifi cantly higher in patients with complete response in comparison with the group with no response, median (95% CI): 47 (35–58) vs 16 (9–20), p < 0.001.Multivariate analysis (logistic regression) was performed. According to this regression, a risk factor for an unfavorable response was detected — males > 60 years of age, p = 0.05; RR (95% CI): 2.0 (0.9–7.1). A predictor of unfavorable response was identifi ed – immediate preoperative platelet count < 23 × 109/l (cutoff point determined in ROC-analysis); p = 0.001, RR (95% CI): 2.5 (1.1–8.6). The probability of complete response was lower with the number of treatment lines prior to splenectomy (weak inverse correlation: r S = −0.30; p = 0.01). The frequency of postoperative complications was 12.6 %. According to our follow-up data, a complete response was preserved in 66/79 (83.5 %) of patients, with a follow-up of 2.7 years.Conclusion. Splenectomy is an effective and safe treatment method for ITP. Factors of unfavorable response were identifi ed: males > 60 years of age and immediate preoperative platelet count < 23 × 109/l. It is safe to perform splenectomy regardless of effectiveness of preoperative splenectomy treatment and platelet count.