Chronic myeloid malignancies that have characteristics of both the myelodysplastic and myeloproliferative disorders allocated to myelodysplastic/myeloproliferative diseases (MDS/MPD) group in 2008 World Health Organization classification. This group includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ringed sideroblasts and thrombocytosis (RARS-T) and unclassified MDS/MPD. Except RARS-T diseases in this group have similar molecular characteristics and clinical manifestations, which require the study of biology, specific molecular markers, morphological features and a clearer definition of nosology. This review provides information on international guidelines for the diagnosis and treatment of MDS/MPD.

Objective: evaluation and comparison of positron emission tomography (PET) prognostic value with other predictors of effectiveness in patients with non-Hodgkin’s lymphoma (NHL) receiving high-dose chemotherapy with autologous hematopoietic stem cell transplantation (AHSCT).

Materials and methods. The retrospective data on 49 consecutive patients with NHL  receiving high-dose chemotherapy with АHSCT was               analyzed. The median age was 36.2 (7–60)  years. Median follow-up is currently 24 (1–82)  months. Prognostic factors analyzed included sex, response to the initial chemotherapy, time to relapse, second-line chemotherapy regimen type, B-symptoms on relapse, serum lactate dehydrogenase and albumin levels, response assessed by computer tomography (CT), number of chemotherapy lines, condition regimen, PET-scan results before (PET1, n = 49) and after (PET2, n = 39) AHSCT.

Results. Two-year overall and event-free survivals were 74.4 and 79.1 %, respectively. Patients with CT-confirmed progression prior to AH-SCT had the worse prognosis. Prognostic significance of PET-status  was shown in chemosensitive patients (partial/complete response).

The overall survival in PET1-negative and PET1-positive patients were 95.4 vs 71.0 % (р = 0.019), respectively. In PET2-positive and PET2-negative patients the overall and event-free survivals were 59.8 vs 100 % (р = 0.001) and 54.4 vs 94.4 % (р = 0.02), respectively.    

In combined analysis of PET1 and PET2 statuses prognostic significance of PET2 prevailed over PET1 results significance. The multivariate analysis confirmed only PET1 significance for survival prediction.  

Conclusion. Chemosensitivity of the tumor, assessed by CT, is the most important prognostic factor. In chemosensitive patients achievement PET1 or PET2 negativity means better prognosis. The patients with PET positivity prior and after AHSCT have the worst prognosis.

ALK-positive anaplastic large cell lymphoma is a heterogeneous group of mature T-cell non-Hodgkin lymphoma, and is characterized by CD30/Ki-1 expression. Recently, value of various prognostic factors is investigated. These include clinical, histological and molecular genetic changes associated with different signaling pathways activation. Some features of the mechanism of action of anaplastic lymphoma kinases and targeted therapies possibilities addressed in this review.

We assessed the prognostic significance of IKZF1 gene deletions in 141 pediatric patients with B-cell precursor acute lymphoblastic leukemia (BCP-ALL)  on Russian multicenter trial in pediatric clinics of Ekaterinburg and Orenburg. IKZF1 deletions were estimated by multiplex ligation-dependent probe amplification. IKZF1 deletions were revealed in 15 (10.6 %) patients. IKZF1 deletions were associated with age older than 10 years (p = 0.007), initial white blood cell count higher than 30 × 109/l (p = 0.003), t(9;22)(q34.q11) (p = 0.003) and delayed blast clearance: М3 status of bone marrow at day 15 of remission induction (p = 0.003), lack of hematological remission at day 36 (p < 0.001) and high levels of minimal residual disease at days 15, 36 and 85 (p = 0.014; p < 0.001; p = 0.001 correspondingly). Patients with IKZF1 deletions had significantly lower event-free survival (EFS) (0.30 ± 0.15 vs 0.89 ± 0.03; p < 0.001) and overall survival (OS) (0.44 ± 0.19 vs 0.93 ± 0.02; p < 0.001), while cumulative incidence of relapse was higher (0.67 ± 0.18 vs 0.07 ± 0.02; p < 0.001). In the multivariate analysis IKZF1 deletions were associated with decreased EFS (hazard ratio (HR) 4.755; 95 % confidence interval (CI) 1.856–12.185; p = 0.001), and OS (HR 4.208; 95 % CI 1.322–13.393; p = 0.015), but increased relapse risk (HR 9,083; 95 % CI 3.119–26.451; p < 0.001). IKZF1 deletions retained their prognostic significance in the intermediate risk group patients (p < 0.001), but not in standard or high-risk groups. Majority of IKZF1 deletions – 12 (80 %) of 15 – were revealed in the “B-other” group (n = 83). In this cohort of patients IKZF1 deletions led to inferior EFS (HR 6.172; 95 % CI 1.834–20.767; p = 0.003) and higher relapse rate (HR 16.303; 95 % CI 3.324–79.965; p = 0.015). Thus, our results showed that IKZF1 deletions are independent risk factor in BCP-ALL patients.

Introduction. AB0-incompatibility in different types of allogeneic hematopoietic stem cell transplantation (HSCT) may be an additional aggravating factor for the development of immunological complications and decrease treatment efficacy.

Materials and methods. From May 1999 to December 2015 in R.M. Gorbacheva Memorial Research Institute for Children Oncology, Hematology and Transplantation 1131 patients with malignancies and hereditary diseases were included to the study, which were performed 1428 allogeneic HSCT: allogeneic unrelated – 814 (57.0 %), allogeneic related – 344 (24.1 %), haploidentical – 267 (18.7 %), umbilical cord blood in 3 patients (0.2 %). Age was 0–76 years, median – 25 years.

Results. In 54.6 % of cases (n = 780) АВ0-incompatibility was determined: major – 37.8 % (n = 295); minor – 45.4 % (n = 354); combined – 16.8 % (n = 131). АВ0-incompatibility in allogeneic HSCT did not influence overall survival (p = 0.56), frequency of acute graftversus-host disease (GVHD) (p = 0.2). There was an increased frequency of acute GVHD in combination with reduced intensity conditioning regimens and АВ0-incompatibility (30.8 %) compared with myeloablative regimens (15.3 %; p = 0.002).

Conclusion. The presence of АВ0-incompatibility is not a limiting factor to perform allogeneic HSCT, however, it demands high quality prophylaxis and sophisticated transfusion therapy to prevent immune complications.

The review bears on basic principles and technologies of next-generation sequencing (NGS), as well as its applications for detection of gene mutations in leukemic cells. We discuss some novel data concerning NGS approach to studies of genetic heterogeneity in myeloproliferative disorders, detection of high-risk genes, including drug resistance mutations, epigenomic changes associated with leukemias, as well as molecular aspects of clonal evolution. A special section concerns basic problems with bioinformatics and adequate analysis of large digital databases obtained with NGS approach. Optimal choice of appropriate software is of utmost importance for adequate retrieval and interpretation of the NGS data.

We developed and implemented in multicenter setting the standardized approach for flow cytometric minimal residual disease (MRD) monitoring in childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL).  Participation of multicenter group reference laboratories in several ring trial studies demonstrated high concordance rate between participants. Successful integration of one additional laboratory in the multicenter group has shown good level of our approach reproducibility. These results will allow implementing MRD detection in stratification system of pediatric ALL treatment protocols of Russia-Belarus multicenter group.

Here we describe and qualitatively characterize the distribution of mononuclear cells isolated from bone marrow aspirate of healthy donors and sorted by their surface CD antigens on a cell microarray by morphology (for 11 donors) and by absence or presence of α-naphthyl butyrate esterase and naphthol AS-D chloroacetate esterase (for 9 donors). These data can be used as a reference for the diagnosis of acute myeloid leukemia using the cell microarray.