Introduction. Despite the success of current therapeutic approaches and suffi cient supply of clotting factor concentrates, adult patients with hemophilia have a high frequency of developing joint pathologies. Furthermore, an increase in life expectancy has correlated with an increase in the share of patients with concomitant somatic diseases, which are characteristic for this population over the age of 40 years.Aim — to analyze the treatment results of adults with hemophilia living in the city of Novosibirsk and Novosibirsk region and evaluate their orthopedic status, types, frequency of bleeding, and comorbidity structure.Material and methods. A total of 93 patients were enrolled in the study (including 86 patients with hemophilia A and seven patents with hemophilia B). The analysis was carried out separately in subgroups of patients according to age: 18–24, 25–34, 45–44, and over 45 years old. Hemophilia type, severity of disease, strategy of therapy, inhibitor status, and features of concomitant pathology were analyzed.Results. A high frequency of spontaneous bleeding was found in all age groups of patients enrolled in the study (39.6 and 34.9 % during 6 and 12 months, respectively). The average frequency of joint bleeding was 4 cases per person per year in the group of patients aged ≥ 18 years.In evaluation of orthopedic status, it was remarkable that there was a signifi cant number of target joints (262, n = 93), which amounted to 2.8 defected joints per patient (the elbow, knee, and ankle joints were the most frequently involved in pathological process). All patients aged over 18 years with severe and moderate form of disease had a musculoskeletal complication with involvement of one or more target joints.The number of diseases per one patient prevailed in the age group over 45 years old and amounted to 4.12 comorbidities per patient. The prevalent diseases in the structure of concomitant pathology in adult patients with hemophilia were chronic viral hepatitis C (43.1 %, n = 42), stage I–III arterial hypertension (27.9 %, n = 26), stomach and duodenum diseases as well as gallbladder pathology (33.3 %, n = 31 and 26.9 %, n = 25, respectively), urolithiasis and chronic secondary pyelonephritis (21.5 %, n = 20).Conclusion. Despite the use of clotting factor concentrates in patients with severe and moderate hemophilia, the frequency of joint hemorrhages remains high, regardless of the age prophylactic replacement therapy was started. Additional measures must be taken to prevent the appearance of critical disease phenotype with frequent spontaneous bleeding.

Background. The prognosis of patients with multiple myeloma (MM) is significantly different depending on the biological characteristics of the tumor substrate, the microenvironment of the bone marrow, as well as factors associated with the patient’s body. Therefore, the search for new reliable and easily identifiable prognostic markers is relevant for the effective management of patients with this disease.The objective of the study was to assess the prognostic value of the study of serum free light chains (FLC) of immunoglobulins κ and λ and their ratio κ / λ FLC in the blood serum of patients with newly diagnosed MM in real clinical practice.Materials and methods. 369 patients with first diagnosed MM (134 men and 235 women) were examined who were hospitalized in the hematology department of the City Clinical Hospital No. 2 Novosibirsk in the period since January 2012 to December 2017. The median age of the patients was 67 (32–82) years. All patients received induction courses of chemotherapy based on bortezomib. The control group consisted of 56 conditionally healthy individuals: 34 women (60.7 %) and 22 (39.3 %) men with a median age of 62 (40–68) years. The concentration of FLC-κ and FLC-λ (mg / L) in blood serum was determined by immunoturbidimetric method on a Hitachi 911 automated biochemical analyzer using the Freelite Human Lambda and Freelite Human Kappa reagent kits (Binding Site, Great Britain).Results. It was found that in patients with MM, the concentration of serum FLC-κ or FLC-λ was statistically significantly higher compared to the control group and varied depending on the type of MM (p <0.001). The diagnostic sensitivity of the quantitative determination of FLC and their ratio for MM was 98.64 %, compared with 94.04 % in a standard immunochemical study. The values of the ratio κ / λ FLC <0.04 or> 65, as well as the concentration of FLC-κ and FLC-λ are higher than the median obtained in the whole group (FLC-κ ≥702 mg / L and FLC-λ ≥493.2 mg / L), correlate with known factors of poor prognosis for MM (with a high concentration of β2‑microglobulin (>3.5 mg / L) (r = 0.461; p <0.001), plasma cell bone marrow infiltration >60 % (r = 0.420; p <0.001), renal failure (creatinine >177 μmol / L) (r = 0.380; p = 0.002), and also with high lactate dehydrogenase activity (>450 U / L) (r = 0.520; p <0.001) and is associated with poor outcomes. The median overall survival in the group of patients with κ / λ FLC <0.04 or >65 was 49 months compared to 76 months in the group with κ / λ FLC 0.04–65 (log-rank p = 0.012).Conclusion. The determination of free FLC in the blood serum of patients with MM can be used to assess the prognosis of their survival. The value of the κ / λ FLC ratio <0.04 or >65 allows us to divide patients with MM into risk groups with significantly different outcomes and can be used to identify patients at high risk who need more aggressive therapy and more detailed monitoring of the response.

Aim. To study incidence and structure of comorbidity in multiple myeloma (MM) patients depending on their age; to determine its effect on overall survival, efficacy, and safety of the first-line therapy in real clinical practice. Materials & Methods. Overall, 369 patients with newly diagnosed MM were enrolled in the trial from January 2012 to December 2017. Among them there were 134 men and 235 women hospitalized at the Unit of Hematology in the Novosibirsk Municipal Clinical Hospital No. 2. Median age of patients was 67 years (range 32-82 years). Results. The analyzed patients were divided into three age groups: the first group of young/middle age (32-59 years) (n = 105), the second group of elderly patients (6074 years) (n = 186), and the third group of old age (> 75 years) (n = 78). In each patient prior to chemotherapy the comorbidity spectrum was identified and CIRS-G, CCI, and MCI comorbidity scores were calculated. Patients with newly diagnosed MM in real clinical practice prove to have high and increasing with age comorbidity incidence (91 % in patients of young/middle age, 97,7 % and 100 % in patients of elderly and old age, respectively). Comorbidity significantly reduces overall survival (OS) of MM patients. Important OS predictors are rhythm and conduction disorder (odds ratio, OR, 2.762; p < 0.002), chronic pancreatitis (OR 1.864; p < 0.001), exogenous constitutive obesity (OR 1.948; p < 0.002), chronic obstructive pulmonary disease (OR 2.105; p < 0.021), chronic kidney disease, stage С4-С5 (OR 2.255; p < 0.003), and chronic heart failure, functional class II (OR 1.915; p < 0.002). Highest importance in predicting OS, efficacy, and tolerance to chemotherapy in MM patients is attached to MCI score (OR 3.771; p < 0.001). MM patients with high risk by MCI are characterized by lower rate and depth of response to the first-line therapy, shorter time before the first relapse, higher incidence of non-hematologic toxicity of grade > 3, and therapy withdrawal or drug dose reduction.

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Background. The annual reporting forms for malignant neoplasms do not provide for the division of lymphomas into variants. In international publications and statistical reference books of the Russian Federation, variants of non-Hodgkin’s lymphomas (NHL) are also not separated. For these reasons, a detailed assessment of the epidemiology of NHL in Russia is difficult, and data for individual variants of the disease are not provided.Purpose: to present the clinical and epidemiological characteristics of Diffuse Large B-cell Lymphoma (DL BCL ) in Novosibirsk according to the data of the City Hematology Center (CHC).Material and Methods. A retrospective analysis of primary medical documentation (case histories,  outpatient charts, and immunohistochemical study reports) of 271 patients with DL BCL was performed in the period from January 1, 2013 to December 31, 2018.Results. The calculated registered primary morbidity and mortality from DL BCL in Novosibirsk for the analyzed years fluctuated slightly. The average incidence and mortality rates were 2.85 and 1.98 per 100,000 population, respectively. When analyzing the dynamics of the prevalence of DL BCL in Novosibirsk, a positive trend of annual increase in the indicator was revealed: 1.87 times over the past 6 years. In men, the tumor developed 2.6 years earlier than in women. In General, the risk of getting DL BCL after 50 years was 3 times higher than at the age of 30 years. Among patients diagnosed with primary lymphoma who underwent treatment at the Hematology department of CHC, half of the patients had DL BCL .Evaluation of the clinical characteristics showed a pronounced severity of the analyzed cohort.Conclusion. The updated quantitative and qualitative indicators of clinical and epidemiological characteristics of DL BCL in Novosibirsk for 2013–18 were obtained. These indicators can be used for further monitoring, development and implementation of measures for the prevention, diagnosis and treatment of this form of hemoblastosis.