Tumor thrombosis of the central venous system in children with embryonal tumors is a rare complication, requiring a comprehensive treatment approach, with chemotherapy and the intervention of a multidisciplinary team of oncologists and cardiac surgeons. The article describes the medical history of a 9-month-old patient with bilateral nephroblastoma and tumor thrombosis of inferior vena cava and right atrium, as well as provides a brief review of the literature. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.

During last years dose-adjusted EPOCH chemotherapy has become the most common international fist-line regimen of treatment of primary mediastinal B-cell lymphoma ^ МВ ^). Our goal is to evaluate results of multicentre international trail of treatment for children with PMBCL according to B-NHL- М - Ю and with dose-adjusted EPOCH protocol. This study was supported by the Independent Ethical Committee of State Nature Research Institute and approved by the decision of the Academic Council. More than 20 clinics from Russian Federetion and republic of Belarus are involved in this reaserch. Since January 2011 to June 2017 246 patients with first diagnosed NHL were included in the study, 18 of them had PMBCL (7.3%). Results: 10 patients (55.5%) were treated according to BNHL protocol, 8 (44.4%) patients received D а -EPOCH-R protocol. With median follow-up of 3-year, EFS for patients with treatment according to B-NHL- М - Ю protocol was 70% for other with D а -EPOCH-R - 67%.

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and clinically aggressive type of T-cell lymphoma that characterized by proliferation in the liver, spleen and bone marrow. The article presents analytical review of literature and the clinical case of γδ HSTCL in 4 years old child. Patient's parents agreed to use personal dats and photos in research and publications.

Introduction. The main features of bone marrow blasts cells in Burkitt lymphoma/leukemia (BL) are L3 morphology, mature immunophenotype of blasts with surface IgM expression, and presence of typical MYC gene rearrangements.The aim of the study was to show discrepancy examples in laboratory signs of BL.Patients and methods. 10 patients (8 boys and 2 girls) aged 1 to 18 years were included in the present study. The inclusion criterion was the identification of discrepancies between flow cytometric, morphological and cytogenetic data.Results. In 2 cases there were no rearrangements of the MYC gene. In 2 patients, the L2 morphological variant went against the presence of typical MYC gene rearrangements. In one case, undifferentiated blasts cells were described by morphology together with presence of surface IgM, and atypical genetics. In 8 patients, there was no expression of surface IgM. Of these, patients with absence of cytomorphological data cytometric and genetic data were controversial.Сonclusion. The cases presented in this study and the cases described in the literature demonstrate the importance of an attentive and comprehensive approach in evaluating the results of laboratory tests in the diagnosis of BL.

Introduction. Surface immunoglobulin expression is a main immunophenotypic criteria of mature subtype of B-lineage acute lymphoblastic leukemia. Although the majority of such cases represents Burkitt leukemia/lymphoma, it was shown for several times that membrane IgM could be detected in the absence of other mature lymphomas signs.The aim of the study was to evaluate heterogeneity of childhood acute lymphoblastic leukemia (ALL) with surface IgM expression and to assess correspondence of BIV EGIL ALL subtype with Burkitt lymphoma (BL) bone marrow dissemination.Materials and methods. Immunophenotypic, cytomorfologic and genetic data of 54 BIV-ALL cases were analyzed.Results. Among the studied patients 39 had BL, while others belonged to B-cell precursor ALL (BCP-ALL). All BL patients and none of BCP-ALL patients carried C-MYC rearrangement while in BCP-ALL group in 8 cases and in any BL cases KMT2A rearrangements were found. None of BCP-ALL children had L3 morphology according to FAB classification.Conclusions. B-lineage ALL with surface IgM expression is rather heterogeneous group of cases including typical BL and rare cases of BCP-ALL even with KMT2A-rearrangements. Combination of all available diagnostic technologies will allow precise split of these two different disease and select the appropriate treatment scheme.

The relapse of acute lymphoblastic leukemia (ALL) in children is still a difficult task. This is due both to the difficulties of achieving a second remission and to the problems of organizing allogeneic bone marrow transplantation (BMT) in those patients who need it. The standard approach is the use of second-line drugs in the regime of high-dosage chemotherapy blocks, but the response rate for this treatment in patients with early relapses remains low and the response time is short. Recently, new drugs with different mechanisms of action have appeared that can give an opportunity for a full remission. The organization of a multicentre study on anti-relapse therapy in the Russian Federation is an urgent task, the solution of which can help in studying new approaches to therapy and in the organization of BMT logistics. This article presents the results of a pilot multicentre study on the treatment of relapses of ALL in children in the Russian Federation. The effectiveness of standard therapy for a group with late relapses and the efficacy and toxicity of interventional blocks with the use of bortezomib, clofarabine, nelarabine are presented.

AIM:A comparative analysis of efficacy and toxicity of two chemotherapy regimens: standard German protocol ALL-BFM 90m and less intensive original test protocol ALL-MB 91 in a multicenter trial of acute lymphoblastic leukemia (ALL) in children.MATERIAL AND METHODS:In 1995-2002 a total of 834 patients with newly diagnosed ALL aged 0-18 years were admitted to 10 clinics of Russia. Of them, 713 were randomized in two groups: treatment program ALL-BFM 90m (n = 355) and ALL-MB 91 program (n = 358).RESULTS:In 7-year follow-up median, 10-year event-free survival (EFS) and overall survival (OS) did not differ significantly between the groups and was 67 +/- 3 and 68 +/- 3% (ALL-MB 91) and 74 +/- 2, 71 +/- 3% (ALL-BFM 90m), respectively. Though the rate of isolated recurrences in CNS in patients on the protocol ALL-MB 91 was 2.8%, they developed only in 0.8% patients of the standard risk group. Anemia, thrombocytopenia and agranulocytosis developed less frequently, hospital stay was significantly shorter on the test protocol vs the control one (p < 0.01).CONCLUSION:EFS and OS on the test (ALL-MB 91) and control (ALL-BFM 90m) protocols were equivalent in lower toxicity and cost of therapy.

Prognosis for children treated according to the BFM-90m protocol (Berlin-Frankfurt-Munster Group) for acute lymphoblastic leukemia (ALL) improved significantly as compared with previous modalities. Methotrexate was used in the dose of 1,000 mg/m2, 36 h. The paper presents the 10-year results for this modification. Patients aged 0-15 years were treated at hematological hospitals of Moscow, other Russian towns and in Minsk, Belarus, (July 5, 1990-November 11, 2000). BFM-90m treatment was given to 682 children out of 1,326 with primary diagnosis of ALL; a comparative trial of the MB-91 protocol hed been carried out at the same clinics since 1991. During 10 years, recurrence-free survival was 72% while overall survival--77%. Toxicity of side-effects was tolerable. The BFM-90m treatment showed significantly better results in both countries.

The report deals with the results of application of an original protocol--the Berlin-Moscow-91 (BM-91)--for the treatment of acute lymphoblastic leukemia (ALL) in children. The researchers' major concern was to improve survival and cut down side-effects incidence as well as to prevent and successfully manage occult neuroleukemia as a potential source of relapse. Patients aged 5 months-15 years received the BM-91 and ALL BFM-90m treatment first at one clinic and later at several centers. Out of 852 children with primary diagnosis of ALL admitted to Russian hematological hospitals (March 2, 1991-November 3, 2000), 687 were included into the study; 329 received the MB-91 protocol. Nine-year recurrence-free survival was 73% while overall survival--80%. Toxic side-effects after L-asparaginase were reported in 27 (7.9%). It is concluded that good results in childhood ALL treatment can be achieved without resorting to high-dosage chemotherapy and radiation in most cases.