Vol 17, No 4 (2018)

Both the ratio of different leukocyte subgroup content and the leukocyte morphology in peripheral blood of newborns are important in diagnosis of several diseases including combined immunodeficiency and neonatal septicemia. There is a need for development of screening methods for parallel study of the leukocyte morphology and population structure in the newborn peripheral blood. We aimed to determine the relative abundance of different leukocyte subsets and to study their morphology in full-term and premature newborn babies and healthy adult volunteers using the cell-binding microarray – a transparent support with immobilized antibodies against leukocyte cluster-of-differentiation antigens. The work was supported by the Scientific council and approved by the ethical committee of the Centre. We have studied the peripheral blood of 12 full-term newborns (38–40 weeks gestation), 9 premature newborns (22–32 weeks gestation) and 18 healthy adults. The relative abundance of the leukocyte and their morphology were determined using the cell-binding microarray including antibodies against CD2, СD3, СD4, CD5, СD7, CD8, CD10, СD11b, CD11c, CD13, CD14, CD15, CD16, CD19, CD20, CD22, CD25, CD33, CD38, CD41a, CD45, CD45RA, CD45RO, CD61, CD64, CD117, CD123, HLA-DR. The percentage of leukocytes positive for every of the studied surface CD antigens among the peripheral blood mononuclear cells of full-term and preterm newborn babies and healthy adults determined on the cell–binding microarray are in good agreement with published flow cytometry data. CD11b+ leukocytes both in premature and full-term newborns included up to 21% myelocytes and 27% metamyelocytes. The reported data can be used as reference values in cell-binding microarray application in diagnosis of combined immunodeficiency or neonatal septicemia.

It presents the results of an epidemiological study of acquired aplastic anemia in children (0–17 years) in the Republic of Belarus for the period 1990–2015, taking into account the condition of the patient's residence area after the Chernobyl nuclear power plant accident. Тhere are 118 districts in the Republic of Belarus, from them 49 are the affected areas and 21 of this 49 are the most affected. A severe incidence rate was 0.317 ± 0.071 per 100 000 children in the areas most affected by the Chernobyl nuclear power plant accident, while in unaffected areas it was 0.217 ± 0.025 per 100 000 children (p > 0.05). The incidence of boys, regardless of the condition of the territory, was always higher than the incidence of girls living in the same territories. So a severe incidence rate of boys was 0.260 ± 0.027 per 100 000, a severe intensive incidence rate of girls – 0.187 ± 0.025 per 100 000 (p < 0.05). For the affected areas these rates were 0.304 ± 0.051 per 100 000 and 0.175 ± 0.040 per 100 000 respectively (p < 0.05). For the most affected – 0.402 ± 0.112 per 100,000 and 0.228 ± 0.086 (p > 0.05). The obtained data showed the uneven distribution of acquired aplastic anemia in the population, depending on sex and the condition of the territory. These data are planned to be taken into account in the course of further work during analysis the results of treatment.

123I-MIBG (metaiodobenzylguanidine) scintigraphy and 99mTc-MDP bone scintigraphy (bone scan) are widely used in diagnosis and staging of NB. Consecutively using both methods lead to overall radiation exposure. As most of the patients need additional anesthesia to perform these diagnostic procedures, which accordingly increase the side effect, workload and expenditure. To minimize the exposure, side effect, workload and expenditure, we set the tasks to optimize the diagnostic algorithm. Determine the indications of bone scan after 123I-MIBG scintigraphy. Materials and Methods: 129 patients with NB were included in retrospective study who underwent both diagnostic procedures at interval of less than 30 days at the stage of initial diagnostic procedure using standard protocols. Out of 83 MIBG positive patients bone scan was able to detect metastatic lesions only in 48 patients. However, bone scan had revealed metastatic lesions in 3 out of 14 MIBG-negative patients. Bone scan is indicated only in MIBG-negative neuroblastoma patients; and in cases where the MIBG uptake cannot be determined for example after operative treatment or after starting of chemotherapy.

The problem of blood-borne infections remains relevant in transfusion medicine. Pathogen reduction technologies (PRT) provide a preventive approach to a wide range of transfusion-transmitted infectious diseases. To date, PRT widely used for a number of blood components, however, the use of these technologies for the treatment of erythrocyte-containing components has not been studied. Objective: to conduct a comparative analysis of the clinical efficacy of transfusions of pathogen-reduced and gamma-irradiated erythrocyte suspension in pediatric patients with various oncological and hematological diseases. Seventy transfusions of red blood cell suspensions (RBC-S) (35 transfusions of pathogen-reduced RBC-S and 35 transfusions of gammairradiated RBC-S) in pediatric patients with oncological and hematological diseases were analized. Clinical efficacy parameters such as the hemoglobin and the hematocrit increment after transfusion, the interval between transfusions, the frequency and severity of transfusion reactions were estimated. We also evaluated the correlation between the hemoglobin and the hematocrit increment with age, patient’s body weight, the hemoglobin concentration and patient's hematocrit before transfusion, the volume of transfusion, the hemoglobin dose and the adjusted hemoglobin dose received for transfusion. We found that the clinical efficacy and safety of RBC-Ss of the compared groups did not differ: the hematocrit and the hemoglobin increment, the frequency and severity of transfusion reactions, and the interval between transfusions were comparable between groups. There was no evidence of immune elimination and allo-sensibilization caused by pathogen-reduced RBC-S. In the group of patients receiving pathogen-reduced RBC-S, a correlation was found between the increase in the hemoglobin and hematocrit values with some of the EV indices. According to our data, the spectrum of efficiency and safety indicators of pathogen-reduced RBC-S is no worse than that of gamma-irradiated RBC-S, provided that RBC-S is used for 14 days of storage.

Family thrombocytopenia/thrombocytopathy with a predisposition to the development of acute myeloid leukemia (AML)
is a rare disease associated with a mutation in the RUNX1 gene. To date, there are data on this disease in no more than 70 families. We present a description of the clinical observation of this pathology in two twin children, and also offer an analysis of available literature on the pathogenetic aspects and prevalence of this rare disease. Patient's parents agreed to use personal dats and photos in research and publications.

Leiomyoma of oesophagus is a rare tumour in adults and even more rare finding in pediatrics. Biopsy or enucleation of tumour is necessary to exclude malignancy, especially in a case of any malignancy symptoms, for example, severe weight loss. Total excision of benign tumour is curative. In our report we present the clinical case of successful thoracoscopic excision of esophageal leiomyoma in adolescent.

The length of the end of chromosomes – telomeres is a dynamic and constant characterizes cellular aging process. Measurement of telomere length (TL) and its connection with the diagnosis, course, prognosis and treatment of a number of diseases in recent years, is of great interest for researchers. The methods used for this are varied and are primarily by molecular biological. Variability of methodological approaches, the lack of a common standard of measurement TL measurements in absolute and relative terms: all this leads to results that cannot be compared with each other. The aim of this work is the development of data conversion algorithm is obtained by flow cytometry in absolute telomere length, expressed in kilobases (kb). The study was conducted from venous blood of children 2–3 years of age (8 healthy children and 3 patients with dyskeratosis congenita). As control material used cell line 1301 (ATCC, UK). Determination of relative telomere length was performed by using a set FlowFISH Telomere PNA Kit / FITC (DakoCytomation, Glostrup, Denmark). RTL code and the absolute length of telomeres were calculated for each study participant. Data on the length of patients telomere were compared with data on TL healthy children of appropriate age. RTL average value for healthy children 2–3 years was 30.1, and an absolute telomere length – 15.56 kb. Telomere length patients with dyskeratosis congenita was significantly shorter (RTL 1.4–4.3, the absolute value of TL 0.3–2.4 kb). The algorithm conversion molecular fluorescence equivalent in absolute TL is universal and can be used in any laboratory equipped with a flow cytometer. The resulting data TL can be compared with the data expressed in absolute units and obtained by any other method.

The hyper-IgE syndrome with dominant-negative mutations in signal transducer and activator of transcription 3 (STAT3) gene is a combined primary immunodeficiency characterized by severe bacterial infections (skin and lungs with bullae formation), characteristic phenotype, serum IgE elevation, eosinophilia, as well as connective tissue, and bone anomalies. Patients also have  high risk of cancer. STAT3 is a transcription factor important for the JAK/STAT signaling pathway, which plays the key role in the synthesis of cytokines, hormones, and bioactive agents. Hyper-IgE syndrome therapy includes antimicrobial prophylaxis, immunoglobulin replacement, and use of bisphosphonates. Hematopoietic stem cell transplantation is an alternative way for the disease treatment. Here we describe a patient with severe autosomal dominant hyper-IgE-syndrome with thte loss-of-function mutation in the STAT3 gene. Patient's parents agreed to use personal dats and photos in research and publications.

The review focuses on the antitumor L-asparaginase preparation, its properties and mechanism of action, as well as its new drug forms, which possess improved characteristics of efficiency, safety and pharmacokinetics. Particular attention is paid to L-asparaginase included in erythrocytes.

Blood clotting disorders are one of the leading direct causes of death in developed countries. Laboratory tests are used to detect abnormalities in the hemostasis system and to monitor drug therapy. Standard tests of hemostasis have a number of limitations, so until now, there is a search and development of new, more informative and sensitive methods. One such device is thrombodynamics, a new method for studying hemostasis by measuring the growth rate of a fibrin clot. This review presents the algorithms for performing the thrombodynamics test and the interpretation of the state of the clotting system in blood plasma using this method, as well as a critical analysis of the existing base of clinical studies of thrombodynamics.