The aim of this study was to determine the prevalence and risk factors of intestinal colonization with extended-spectrum beta-lactamase producing Enterobacteriaceae (ESBL-E) in patients with acute myeloid leukaemia (AML) and lymphoma.
Material and methods. 98 patients (median age of 43 years; 33 – AML, 65 – lymphoma) were included in the prospective study. Follow-up period was lasted for 6 months. Rectal swabs were collected within 2 days of admission and every 7 days further. Presence of ESBL was confirmed by phenotypic tests, genes bla^TEM and bla^CTX-M were detected by PCR. A total of 88 ESBL-E were isolated in 75 (76.5%) patients.
Results. Genes bla^CTX-M were detected in 69% of isolates, bla^TEM – in 49%, both genes – in 36%. Probability of intestinal colonization with ESBL-E was 91% in patients with lymphoma and 84% - in AML. Probability of persistent ESBL-E carriage was 30.3%. Recurrence of ESBL-E carriage was in 13 (39%) of 33 patients with a median of 37 days. Probability of recurrent ESBL-E colonization was 49.4%. Risk factors for ESBL-E carriage proved to be parenteral nutrition (p = 0.05) and permanent hospital stay (p = 0.002). The median of permanent hospital stay in these patients was 70 days (64–180 days). The rate of ESBL-E bacteremia in patients colonized with subsequent microorganisms was 7% (5/75). No bacteremia cases were in patients without colonization. Patients with AML and lymphoma underwent chemotherapy had a high risk of ESBL-E intestinal colonization. The presence of ESBL carriage was not permanent in all patients for 6 months. ESBL colonization should be considered in choosing antibiotics for prophylaxis and empirical approaches in patients with febrile neutropenia.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) nowadays allows cure malignant and nonmalignant diseases considered to be fatal previously. The number of cancer survivors’ children progressively increases over years. Unfortunately, numerous late effects of allo-HSCT become both a serious problem and a cause of the morbidity and mortality. Among them the reproductive pathology seems to be frequent, and fertility problems influence the quality of life of patients significantly.
Material and methods. We present the results of the evaluation of reproductive system in 294 children after allo-HSCT. Reproductive problems were revealed in 43 children and adolescents (15% of all patients and 46% of the patients with endocrine pathology). Hypergonadotropic hypogonadism was the dominating problem (33 patients, 77% of all reproductive dysfunctions).
Results. Risk factors of its development included: busulfan-containing conditioning regimen (р < 0.001, risk ratio (RR) of hypogonadism was 26.7% compared to 5.2% in other regimens), diagnosis of the malignant disease (р = 0.002, RR 26.6% in malignant and only 8.1% in nonmalignant pathology), central nervous system irradiation prior to HSCT (p = 0.002, RR 32.6% with cranial irradiation compared to 15.6% without it), female gender (р = 0.003, RR increases up to 3 times – 26.9% in girls against 9.3% in boys). We also describe cases of the fertility restoration and birthgiving. The algorithm of diagnostic and follow-up of gonadal pathology in children after allo-HSCT is shown.

Cardiovascular diseases (CVDs) are the major cause of worldwide mortality. The most common CVD is essential hypertension (EH), which is registered in 40% of the adult population and 2.4–18% of children and adolescents in Russia. The study of hemostatic status in hypertensive patients indicates to the prothrombotic changes (PTC). Both EH and hemostatic disorders are known to be heredity associated. However factors related to PTC in adolescents with EH are not clearly defined.
The aim of the study was to evaluate factors potentially increasing the frequency of PTC in adolescents with EH.
Material and methods. The heritability, indices of blood pressure monitoring (BPM), hemostasis disorders and thrombophilia gene polymorphisms are studied in 97 Caucasian adolescents (aged of 14–17 years) with EH, living in the Eastern Siberia. 60 adolescents had no PTС (1st clinical group), 37 adolescents had PTС (2nd clinical group). Forty healthy age- and gender-matched adolescents are controls.
Results. We revealed an important contribution of strong family history of thrombosis, stable arterial hypertension, thrombinemia, C/T and T/T allele carriage С677Т MTHFR gene to the development of PTC in adolescents with EH.
Conclusion. Bearing in mind multifactoriality hemostatic changes in pediatric patients with cardiovascular diseases, the further researches in this are necessary.

Material and methods. 148 patients (69 males, 79 females) with multiple myeloma (MM) and cardiac pathology were included in the study during March 2008 – May 2010. The median age of patients was 64.7 years (ranges 36.3–82.7). The patients were divided in two groups. In the first group the patients with de novo with MM (n = 72) were included. The patients with relapsed or refractory MM (RR; n = 76) were included in the second group.
Results. The bortezomib-containing regimens (VCD, VMP or VD) were used as anti-myeloma treatment. The overall response was documented in 65.7% and 59.5% cases including complete (CR) and strong complete remission (sCR) in 22.9% and 20.3% cases respectively. For a median follow-up of 4.9 years for the comparison groups, the 5-year overall survival (OS) was 22.8 ± 5.3% and 17.3 ± 4.4% (p = 0.295). The median OS was 40 and 31.8 months respectively. In multivariate analysis only ECOG scores ≥ 2 were demonstrated an independent negative prognostic value both for the event-free survival (Hazard ratio 1.69; p = 0.006) and OS (Hazard ratio 1.76; p = 0.003).
Conclusion. The bortezomib-based treatment of MM patients with concomitant cardiac pathology was not accompanied by an increase in the incidence of cardiovascular adverse events.

Material and methods. Sequence based typing was used to identify human leukocyte antigen HLA-A, HLA-B, HLA-C, HLA-DRB1, HLA-DQB1 alleles from 403 recruited volunteers in Irkutsk region (Buryats), Russia for unrelated hematopoietic stem cell registry.
Results. During the carried out research in the study population identified new allele, which were registered by The WHO Nomenclature Committee for Factors of the HLA System. In this population, 31 – HLA-A, 58 – HLA-B, 33 – HLA-C, 42 – HLA-DRB1, 19 – HLA-DQB1 alleles were selected. Allele frequencies more than 10% included HLA-A*24:02 (22.08%), HLA-A*02:01 (13.65%), HLA-A*01:01 (10.92%), HLA-B*40, HLA-C*03:04 (17.62%), HLA-C*06:02 (15.51%), HLA-DRB1*07:01 (13.65%), HLA-DRB1*04:01 (10.92%), HLA-DQB1*03:01 (29.9%), HLA-DQB1*02:02 (11.29%). Of the 425 five-locus HLA haplotypes were determined with software Arlequin v.3.1. The highest frequency extended five-locus haplotype – A*24:02-B*40:02-C*03: 04-DRB1*04:01- DQ*03:01 was observed with frequencies of 3,27%. Distribution of allele and haplotype analysis allowed to compare the population of Buryats with other Russian populations. Deviations from the Hardy-Weinberg law for the loci of HLA-A and HLA-DRB1 are revealed.

Presence of Serum-Free Immunoglobulin Light Chains (sFLC) was analyzed for 15 patients with newly diagnosed Chronic Lymphocytic Leukemia (CLL). All patients were also characterized by peripheral blood lymphocytes’ immunotype (including κ/λ), presence of absolute lymphocytosis and clinical data. Of the 15 patients 11 had elevated concentrations of monoclonal sFLC: 7 patients had elevated κ sFLC (κ/λ ratio > 1,65); 4 patients had elevated λ sFLC (κ/λ ratio < 0,26). The type of elevated clone for sFLC was the same as the isotype of clonal lymphocytosis. The remaining 4 of 15 patients did not have elevated concentrations of sFLC whereas clonal lymphocytosis (λ type for all 4 patients) and absolute lymphocytosis were observed. 10 of 15 patients received 6 courses of RFC-therapy. For those patients sFLC concentration was measured before and after the therapy. In 6 of 10 patients tumor clone of sFLC was detected. After RFCtherapy, 4 of those patients demonstrated a reduction in concentrations of elevated sFLC on average for 63,1% and a normal κ/λ ratio that corresponded with the absence of clonal and absolute lymphosytosis. The remaining 2 patients did not have a decrease in sFLC concentrations and had abnormal κ/λ ratio, however, tumor clone lymphocytes and absolute lymphocytosis were not detected, that was presented with better overall clinical outcome. In 4 out of 10 patients that demonstrated no initial elevation in clonal sFLC, measured sFLC concentrations varied within the normal range. Conclusions: 73,7% of CLL patients in our study showed elevated concentrations of clonal sFLC. Serum-Free Light Chains concentration could serve as an integral criterion for evaluation of tumor mass and therapy effectiveness.

Thrombocytopenia is a symptom of a wide range of hematological and non-hematological diseases, requiring extensive diagnostic search. Rapid detection of the main cause of thrombocytopenia affects the prognosis and therapeutic tactics in a number of patients with hematological diseases. One of the reasons for the development of secondary thrombocytopenia are primary immunodeficiency disorders (PID), whose debut is possible at the age of over 18 years. Diagnosis of this pathology is difficult, due to the low alertness of primary care physicians about the presence of favorably occurring primary immunodeficiencies, the first manifestation of which can be the immune thrombocytopenia revealed in an adult. This article is devoted to the report of the clinical cases of newly diagnosed PID in adulthood, debuting from autoimmune complications.

The case report of a rare disseminated invasive mycosis due to Saprochaete capitata in a patient with acute lymphoblastic leukemia (ALL). The main risk factors were prolonged granulocytopenia for 21 days, presence of a central venous catheter (CVC), refractory form of ALL. S. capitata was isolated from blood cultures and CVC. Invasive mycosis due to S. capitate developed in patient receiving echinocandin for 5 days and was represented by septic shock at the onset, early dissemination in the organs (liver, spleen, kidney), prolonged fever for 167 days. Clinical improvement was achieved by using the combination of voriconazole with amphotericin B and removing of CVC. Total duration of voriconazole treatment was more than 200 days, of those 101 days in combination with amphotericin B. ALL treatment was continued with concomitant voriconazole therapy after improvement of patient condition. Relapse of invasive mycosis did not occur.