Objective: To analyze the long-term efficacy and safety of ATR in adult patients with primary resistant ITP in real-world clinical practice. Materials and methods.The article contains long-term results analysis of ATR application under real clinical practice conditions in 138 patients (40 men and 98 women) whose median age at the beginning of therapy was 59 (18-86) years. Two ATR medicines-romiplostim (100 patients) and eltrombopag (38 patients) were used. Results. During the first month of therapy, the median platelet count in the romiplostim group increased from 17·109 / L to 60·109 / L (9-600·109 / L), and the elethrombopag from 16.109 / L to 56.109 / L (9-400·109 / L). The minimal response (reaching platelet counts over 30·109 / L) was achieved in 92% of cases in both groups. Partial response (achievement of platelet count more than 50·109 / L) was achieved in 91 and 84% of patients in the rhombostim and eltrombopag groups, respectively. The frequency of complete response (an increase in platelet counts above 100·109 / L) was noted somewhat more often in the rhyploistim group-69% compared to 47% in the eltrombopag group (P = NS). Most patients demonstrated a long-term stable effect in the form of an increase in blood platelet count to a safe level during months and years of ATR treatment. The achievement of at least partial remission for 3 months or more was 70 and 71% in romiplostim and elthrombopag groups, respectively. Patients who started ATR- therapy are currently continuing treatment: 51% - in romiplostim group and in eltrombopag group-39%. The main reason of discontinuation the initially effective therapy were the loss of platelet response, toxicity, withdrawal from treatment (withdrawal with preservation of remission) and patients death. The tolerability of drugs with long-term admission was satisfactory. The most common AE were headache, bone pain, thrombosis, increased blood pressure and petechial hemorrhagic eruptions. The overall incidence of complications did not differ significantly between the romiplostim and eltrombopag groups -15.6 and 15.8%, respectively. Conclusion. Long-term ATR-therapy using in patients with resistant chronic ITP is an effective and largely safe treatment option.

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Aim. To study the epidemiology of multiple myeloma in the city of Moscow and compare the results obtained with data from similar studies in other countries. Materials and methods. The study is based on information from a database of case histories of 3942 patients suffering from symptomatic MM, residents of the city of Moscow, which is maintained at the Hematologic Moscow City Center of S.P. Botkin Municipal Clinical Hospital. The control of the completeness of inclusion was carried out by cross - comparison with the data of the Moscow Cancer Register and the Register of Program 7 (beginning in 2019 - 12) of Highly Expensive Nosologies. The assessment was made according to data as of January 1, 2019. The calculations were carried out taking into account the data of Rosstat at the beginning of 2019 on the population of Moscow in different gender and age categories. Results. Among the 3942 patients with active MM 1707 men - 43% and 2241 women - 57%, the median of the current age was 68 (28-94) years. The median time of observation of patients since the diagnosis of the disease 34 (1-423) months. The peak incidence was in the age range of more than 60 years. There were no significant differences in gender ratio in different age strata with a breakdown of 10 years. The number of cases of newly diagnosed MM per year for the period from 2009 (n=219) to 2018 (n=385) increased by 75.8%. At the same time, the demonstrated increase in the incidence rate for the described period turned out to be fair only for groups of patients over 50 years old, with the maximum increase in this indicator over the described period in the age range of 60-69 years. This is mainly due to the increase in life expectancy in Moscow in recent years. The study demonstrated that over the past 10 years, the average annual mortality rate from MM has decreased in Moscow, and as a result, its prevalence has increased. The rate of 2-year overall survival of patients with MM was 76%, 5-year - old - 49%, 10-year - old - 27%. The median overall survival of patients under the age of 65 when diagnosing the disease was 79 months, and 48 months. The distribution of patients within international classifications was consistent with international data. Conclusions. The study revealed a significant dynamic of the epidemiological situation concerning MM in Moscow. Over the past 10 years there has been an increase in the incidence of MM, as a result of an increase in the life expectancy of the population. The use of modern diagnostics and therapy of MM in real clinical practice has led to a significant reduction in mortality. Due to these factors, an increase in the prevalence of MM in Moscow has taken place, and this process will no doubt progress in the future.

Background: Renal failure (RF) is a common complication of multiple myeloma found in 25% at diagnosis and in 50% during the course of the disease. RF persistence after induction treatment may constitute a factor of ineligibility for autologous stem cell transplantation (ASCT). However, its impact on toxicity and survival post-ASCT is still subject to discussion. Aims: We aimed to compare the toxicity and survival after ASCT in newly diagnosed multiple myeloma (NDMM) patients (pts) with or without RF at diagnosis Methods: Retrospective comparative study of two groups of NDMM pts without RF at diagnosis (Group 1: G1) or with RF at diagnosis (Group 2: G2). These patients were treated according to the Tunisian national referential by dexamethasone, thalidomide +/ -bortezomib and transplanted at the hematology department of the national center of bone marrow transplant between January 2011 and December 2016. The IMWG 2014 and IMWG 2016 response criteria were used to assess hematologic and renal response respectively. Results: Data from 134 patients were analyzed. Median age at ASCT was 54 years (range, 25-65). Sex ratio was 1.2. In all, 114 (85%) and 20 (15%) pts were included in G1 and G2 respectively. In G2, 13 pts (65%) had severe renal impairment at diagnosis; 2 of them required at least one dialysis session. After induction, renal response was complete (70%), partial (15%) or minor (15%). As a pre-transplant conditioning, all pts in G1 received melphalan (MEL) at the dose of 200 mg/m 2 . In G2, pts received either MEL 200 mg/m 2 (85%) or MEL 140 mg/m 2 (15%).

Background. For many years the primary aim of treatment strategy for ph-negative myeloproliferative neoplasms has been to restrain disease progression, with lasting relief and management of symptoms to improve patients’ quality of life. Generally, this did not lead to a significant increase in life expectancy with primary myelofibrosis and didn’t decrease the risk of fibrosis in patients with polycythemia vera and essential thrombocythemia. To date a new class of targeted drugs has been developed, it is JAK2 inhibitors with pathogenetic effects. The results of clinical trials showed the high efficacy of the first registered drug of this its kind – ruxolitinib – that includes a faster reduction in the symptoms of tumor intoxication and in symptoms associated with the development of splenomegaly and increase in the overall survival rates. It is known that the data obtained during clinical trials of medicines may differ from the results obtained in routine clinical practice. In actual practice drugs are used in a much wider heterogeneous population of patients, less limited first of all by age and comorbid characteristics. It is possible to analyze cohorts of patients including a larger number of clinical cases with a longer follow-up period. In this regard of great interest is the actual clinical experience of long-term use of ruxolitinib in patients whose set is limited only by clinical contraindications for prescribing the drug.Aim. To present our own actual experience of targeted therapy of myelofibrosis and compare the results obtained with the data of clinical trials.Materials and methods. Our analysis includes data from 141 patients (67 (47.5 %) men and 74 (52.5 %) women) in a chronic phase myelofibrosis. All patients received ruxolitinib. Of these, 109 (69 %) patients had primary myelofibrosis, 26 (16 %) – postpolycythemia myelofibrosis, 6 (4 %) – postessential thrombocythemia myelofibrosis. The median age at the start of therapy was 62 (18–84) years. The median disease duration before ruxolitinib was prescribed – 79 (1–401) months. According to the dIpSS (dynamic International prognostic Scoring System) criteria, 13 % of patients were assigned to the low risk group, 38 % – to the intermediate-1, 36 % – to the intermediate-2, 13 % – to the high risk group. Most patients (52 %) had grade 3 bone marrow fibrosis.Results. The median duration of treatment was 18 (range from 1 to 115) months. Symptoms of intoxication were relieved 74 (81 %) of 91 patients, the spleen size decreased in 81 % of patients (the spleen size returned to normal in 25 % of patients). The increase in the median hemoglobin level was 15 %. The proportion of patients requiring blood transfusion decreased by 4 times (from 39 to 9 %). Mean platelet levels normalized in most patients with baseline high and low platelet levels. A complete clinical and hematological response was achieved in 16 % (n = 23) of cases, a partial response – in 26 % (n = 37) of cases, clinical improvement – in 21 % (n = 30), disease stabilization – in 33 % (n = 46) of cases. No response was received in 1 (1 %) patient and in 3 (3 %) cases there was progression of the disease. At the time of analysis, 81 (57 %) of 141 patients were continuing the ruxolitinib treatment. The fatal outcome in 33 (22 %) patients was associated with concomitant diseases, among which 20 (14 %) died from proven COvId-19 infection. Overall survival: 1-year 81 %, 2-year 73 %, 5-year 50 %. Overall survival excluding deaths due to COvId-19: 1-year 92 %, 2-year 85 %, 5-year 70 %. Massive splenomegaly and a high degree of fibrosis were unfavorable predictors of prognosis of overall survival.Conclusion. Target therapy with Janus kinase inhibitor ruxolitinib has demonstrated high efficacy in patients with myelofibrosis in routine clinical practice. The most rapid effect ruxolitinib had on the spleen size and the symptoms of intoxication. Tolerability of ruxolitinib therapy was generally satisfactory. The overall and progression-free survival rates in patients with myelofibrosis, receiving ruxolitinib in the clinical setting was consistent with the results of international multicenter clinical trials.