Aim. To compare the efficiency of the treatment of myelodysplastic syndrome (MDS) with azacitidine and cytarabine on the basis of the following criteria: overall survival (OS), toxicity, length of the hospitalization period, the life quality.

Methods. Research includes 36 patients with MDS aged from 42 up to 83. All patients were divided into two groups. The first group includes 15 patients aged from 55 up to 83. Basic therapy of the said patients involved usage of one of protocols, containing cytarabine “7+3” or “small doses of cytarabine” (“SDC”). The second group is represented by 21 patient aged from 42 up to 81. Treatment protocol of this group included azacitidine (Vaydaza) in a dose of 75mg/m² subcutaneouslywithin 7 days. Evaluation of treatment efficiency was held after 2, 4, 6 courses. The treatment was continued until symptoms of disease progression appeared.

Results. Including of azacitidine in treatment of MDS patients demonstrated the advantage in overall survival (38% vs 26.6%; р= 0.038), resulted in prolonged median survival (13.7 vs 7.9 months; р= 0.03). The frequency of the general response while using azacitidine in comparison to SDC constituted 76% vs 53%. The period of the disease until transformation to AML increased from 6.16 months to 10.6 months.

Conclusion.The including of azacitidine in the treatment of MDS patients improved the quality of life, decrease the dependence on blood transfusion, LOS.

Research was carried out on a representative sample of hematopoietic stem cell/bone marrow donors of the registry of the National Research Center for Hematology. According to self-assessments donors were the ethnic Russians. The distribution of HLA-allele group in HLA-A*/B*/C*/DRB1*/DQB1* loci generally was found to correspond to their distribution in other European populations, but has some specific features. These features include: the high frequency of HLA-A*25-B*18-C*12-DR*15-DQB*06 haplotype which is also specific for the other Russian populations and Poles; and the high frequency of HLA-haplotypes probably reflecting the contribution of Finno-Ugric and some Turkic components to the gene pool of Russians. The donors of registry of National Research Center for Hematology (Moscow) are the most closely related to the Russians of Chelyabinsk and Novosibirsk regions, and Poles. These donors are also more differ from Tatars and Bashkir donors, that points the need of the establishment of registries of hematopoietic stem cell/bone marrow donors in different ethnic regions of Russia. The probability of finding of a compatible donor (in case of allogeneic hematopoietic stem cells/bone marrow transplantation) for carriers of some HLA-haplotypes which are common in the Russians is higher in Russian registries than in foreign ones.

We report our experience of 675 (616 primary, 59 revision) joint replacements in 425 patients performed in one hospital. There were 383 patients with hemophilia A and 42 patients with hemophilia B (age from 18 to 76 years). Inhibitor was detected in 18 hemophilia patients. 90% of patients had positive antibodies to hepatitis C (anti-HCV+), 3 patients had HIV infection. There were 552 knee, 115 hip, 5 shoulder and 3 elbow replacements. 23 knee and 3 hip replacements were performed in hemophilia patients with inhibitor. The average follow-up of implants was 6 years (from 4 months to 20 years). There were revision replacements in 8.8% of cases: 5.5% patients with aseptic loosening and 3% patients – with deep infections. In hemophilia patients deep infection occurred much more frequent – 11%. The significantly higher rate of complications (11%) was observed in patients with inhibitors. In spite of elevated risks for the development of complications in hemophilia patients joint replacement is the only effective procedure in the treatment of end-stage arthropathy. Hemophilia patients need an individualized approach in providing hemostasis and prophylactic antibacterial therapy.

Purpose. To investigate clinical features of the extramedullary myeloma and evaluate the efficacy of the high-dose chemotherapy with autologous stem-cell transplantation (autoSCT).
Material and methods. 18 patients (9 men and 9 women) aged of from 23 to 73 years with de novo multiple myeloma were included in the study. All patients had the multiple myeloma with plasmacytomas, 15 patients had soft-tissue plasmacytomas and 3 patients had the extramedullary disease with the involvement of the liver, stomach, submandibular region.
Results. Clinical features of soft-tissue plasmacytomas and the extramedullary disease in de novo multiple myeloma are characterized by a reduced rate of the achievement of a complete response. At the same time, the use of the high-dose chemotherapy with autoSCT allows get the complete remission in some cases. The overall anti-tumor response (complete response + very good partial response + partial response) after the induction phase of the treatment was achieved in 46.6% of soft-tissue plasmacytomas patients. 10 patients from this group underwent ASCT. All treated patients are alive nowadays, the observation period from the time of diagnosis ranged from 12 to 57 months (median 36 months). 2 patients with the extramedullary disease received ASCT, but a complete response after the transplantation failed to persist for a long time, the systemic relapse developed 3 and 18 months later. 2 patients with the high Ki-67 new extramedullary foci were found to appear against the background of the specific therapy, or almost immediately after the transplantation. The presence of the high CXCR4 expression may be a beneficial factor for the remission without extramedullary relapse with plasmacytomas.

The aim of the study was to assess the efficacy of the phototherapy (UVA1 therapy and PUVA therapy) for the resistance to glucocorticosteroid and immunosuppressive treatment of cutaneous form of chronic graftversus-host disease (chGVHD). The analysis of results of the phototherapy was performed. Twelve patients were included ih the study (2 patients with lichenoid type and 10 patients with scleroderma-like type (n = 10) of chGVHD. The patients were treated with the phototherapy including UVA1 therapy (n = 9), PUVA therapy (n = 1), PUVA therapy and UVA1 therapy stepwise (n = 2). In 10 patients the phototherapy was combined with steroid and immunosuppressive therapy, 2 patients received phototherapy as a monotherapy. 10 (83%) patients responded to the phototherapy. The reduction more than 50% of skin lesions achieved in 4 (33%) patients. Reduction of the affected area on 15–50% was observed in 6 (50%) patients and was identified as the clinical improvement. There was no effect of the treatment in case of the reduction less than 15% of skin lesions and that was observed in 2 patients. Skin inflammation (erythema, maculopapular rash) and sclerosis decreased under the phototherapy. Progression of cutaneous manifestations in chGVHD was interrupted in 7 (58%) patients. Reduction or withdrawal of steroid or immunosuppressive therapy due to phototherapy was achieved in 4 patients. Tolerability of UVA1 therapy and PUVA therapy was sufficient, and no adverse effect was observed. Patients were followed for 1 to 12 years (average: 4 years). One patient died due to the relapse of hematological disease, two patients died due to severe complications of chGVHD, others 9 patients are alive. No progression of cutaneous manifestations of chGVHD was observed in 6 (67%) alive patients. None of the patients developed skin cancer after phototherapy, even after several courses of treatment including a large number of procedures (60–136 procedures). Conclusion UVA1 therapy and PUVA therapy are effective methods of the treatment for patients with cutaneous manifestations of chGVHD which are resistant steroid and immunosuppressive therapy.

The development of hematological malignancies is often associated with the influence of various genotoxic unfavorable factors, in particular, with the effect of ionizing radiation. The case report of the acute leukemia in a patient suffered from an accident at the Chernobyl nuclear power plant thirty years ago is presented. The average absorbed dose was estimated from the dicentrics frequency in peripheral blood lymphocyte culture and was 4.3 Gy. At a subsequent long-term clinical observation (27 years), moderate transient instability of hematological parameters was revealed: lymphocytosis, leukopenia and thrombocytopenia, which were associated with chronic persistent hepatitis. Three-color FISH-staining of chromosomes of cultured peripheral blood lymphocytes through 30.1–30.5 years after irradiation exposure demonstrated the level of translocations significantly in 42–54 times higher background value. In 2014, the patient underwent transurethral resection of the prostate in connection with prostate cancer. In May 2015, a course of radiation therapy with a total local dose of 35 Gy was conducted. In December 2015, there was a general weakness. Cytopenia was noted (RBC 3.56 × 1012/l, WBC 2.9 × 109/l, platelets 90 × 109/l). Diagnosis: acute myeloid leukemia, transformation from myelodysplastic syndrome. An abnormal complex clone was detected in 38% of metaphases by the mFISH-method along with other chromosome rearrangements. The patient died due to pneumonia in March 2017.

Clinical observations of plasma exchange therapy in patients with thrombotic thrombocytopenic purpura (TTP) are presented. Difficulties in the early diagnosis and the role of the early use of the pathogenic treatment are considered. The impact of extracorporal therapy in patients with TTP is discussed.

Additional chromosome rearrangements in patients with chronic myeloid leukemia (CML) may point to the unfavorable prognosis and the progression of the disease. The aggressive course of the myeloid hematological malignancies may be associated with the change of the activity of EVI1 oncogene located in the long arm of chromosome 3 and involved in the rearrangement. The CML prognosis is very unfavorable, when structural aberrations include position of this gene. The case report shows the rare secondary translocation t(3;7) (q26;q21) in Philadelphia-positive CML patient with blast crisis. The standard cytogenetic research methods could reveal additional chromosome rearrangements associated with disease progression.

The case of de novo acute promyelocyte leukemia in a 22 year old pregnant woman (gestation age 24–25 weeks) is described. Two courses of “7 + 3 + ATRA” chemotherapy were performed. Molecular remission was achieved after the first induction chemotherapy. A month after the second consolidation chemotherapy at gestation age 36 weeks a living premature fetus was delivered by means of Cesarean delivery. After the delivery two more consolidation courses and supportive chemotherapy were performed. The chemotherapy was finished, the woman for 4 years has been on remission. The child is growing up and shows normal development.

N.G. Kartashevskiy served in the Vladivostok naval hospital only 6 years (1933–1939). However, during these years, he managed to create a new direction in medical support of the Pacific Fleet, which became the prototype of the military transfusion and played a crucial role in the successful treatment of the wounded cases during the Khasan border conflict. The first successful experience of the Blood Service during the fighting showed its high efficacy in acute blood loss cases, shock conditions, in the case of healing complications of wound due to anaerobic infection. The combat experience of Blood Service of the Vladivostok Naval Hospital was used in the creation and organization of the work of such services in all military hospitals of the Armed Forces, later fully confirmed its viability.