Data on treatment results and long-term toxicity of patients with Hodgkin’s lymphoma in Russian Federation are scarce. We present analysis of the S. P. Botkin Hospital based registry. Six hundreds nine patients were identified since 01.01.2006 to 31.12.2015, among them there were 374 (61 %) women and 235 (39 %) men. The median age was 31 years (range, 15–85). One hundred sixty four patients (29 %) had Cotswolds stage IV, 128 (22,5 %) – stage III, 256 (45 %) – stage II, and 20 (3,5 %) – stage I. Among first-line regimens ABVD was received by 26 % of patients, AVD – 1,2 %, COPP/ABVD – 5,6 %, BEACOPP-14 – 22 %, BEACOPP-21 – 19 %, BEACOPP escalated – 2,8 %, EACOPP – 2,6 %, ABVD-BEACOPP – 2,9 %. Sixty seven (11 %) patients received other regimens, including MOPP-ABVD, LABO, LOPP, CEA/ABVD, CHOP and irradiation only. Radiotherapy was given to 81 % of patients. Long-term remission after first-line therapy was achieved in 432 patients (75,2 %). Second-line treatment was required in 117 patients (20,4 %). Twenty five patients (4,4 %) died before second-line therapy. High dose therapy with autologous stem cells rescue (HDCT-ASCT) was conducted in 26 of 117 (22 %) patients with relapse after first-line treatment. Five-year overall survival in early stage patients, receiving ABVD was 96 % and 85 % in those, receiving other regimens. In patients with advanced stages no differences in overall survival were found when comparing BEACOPP
modifications with other regimens. Time to next treatment (TTNT) was different: 5-year TTNT rate was 71 % in BEACOPP group compared to 56 % in patients, receiving other regimens. BEACOPP-escalated and BEACOPP-14 had an advantage over BEACOPP-21: 5-year TTNT rate was 80, 77 and 63 %, respectively.
Long-term toxicity was analyzed by comparing HL patients with age and sex-matched control group, consisting of patients, hospitalized to S. P. Botkin City Hosiptal betwen 01.01.2008 and 01.01.2009 for trauma or infection (n = 555). Standardized incidence ratio (SIR) of developing secondary malignancies was 3,56. SIR of developing all cardiac events, including myocardial infarction, heart failure or cardiac arrhythmia was 2,97. The implications of these findings suggests several ways in which quality of care of HL patients can be improved. These measures include widespread use of PET for precise staging, improvement in integrated care pathways, education of patients, the abandonment of radiotherapy or reduction of volumes and doses of irradiation, greater use of HDCT-ASCT and targeted agents.

Introduction. Cure rate of patients with Hodgkin lymphoma (HL) is high by present. Further survival increase can be achieved by reducing mortality from late complications primarily from postmetahronous malignancies. Primary multiple malignant tumor become a problem for all cancer, including HL.
Materials and methods. The study included 2137 patients with primary I, II, IIIs, IV (epiphrenic lesion) stages HL who received treatment at the Medical Radiological Research Center from 1968 to 2013 years. The diagnosis was verified morphologically. At the time of treatment start the patient age was 13–69 years, before 30 years of age were 1609 (75.3 %) patients. Women were 1412 (66 %), men – 725 (34 %). The main condition for patient inclusion was the amount of irradiation, limited lymph areas above the diaphragm and spleen. Patients were divided into 3 groups depending on method and period of treatment. 1st group – 363 patients received treatment from 1968 to 1977 consisting of independent radiation therapy (RT) according to “radical” program
– irradiation of lymphatic collectors above the diaphragm and spleen (in case without splenectomy) with 40 Gy total focal dose. 2nd group – 1426 patients received treatment from 1978 to 1998 consisting of radiochemotherapy. Chemotherapy (CT) was performed according to COPP, CVPP. RT volume and total focal dose were identical to those of independent RT according to “radical” program. 3rd group – 348 patients received treatment from 1999 to 2013 consisting of CT according to first-line scheme and RT of lesions and related areas with reduced total focal dose (20–30 Gy) exposure.
Multifield RT was used. To calculate the incidence of postmetachronous malignancies counted the number of patients/years of observation after HL treatment by age-appropriate five-year intervals and depending on gender. Incidence of malignant tumors in Russian population obtained from journal “Bulletin of the NN Blokhin Russian Cancer Research Center”. The expected incidence of postmetachronous tumors in HL patients calculated in each age group according to gender. The relative risk of  postmetachronous malignant tumors in HL patients was determined by the ratio of observed to expected incidence. 95 % confidence interval is calculated by J.  Vandenbroucke method.
Results. Follow-up duration after HL treatment was 6 months–36 years (median – 18 years) in the 1st group, 7 months–28 years (median – 14 years) in the 2nd group and 1–17 years (median – 7 years) in the 3rd group. The number of patients/years of observation was 5562 in the 1st group, 13387 – in the 2nd group and 1906 – in the 3rd group. Postmetachronous malignancies were diagnosed in 87 (4.1 %) from 2137 patients: in 27/363 (7.4 %) in the 1st group, in 53/1426 (3.7 %) – in the 2nd group and in 7/348 (2 %) – in the 3rd group. One postmetachronous malignant tumor diagnosed in 81 patients, in 5 patients – 2 tumors and in 1 patient – 3 tumors  appeared consistently. Total number of postmetachronous malignancies was 94 (33 – after radiotherapy, 61 – after chemotherapy), from which solid tumors were 90
(95.7 %) and leukemia – 4 (4.3 %). Time of tumor diagnosis after HL treatment ranged from 1 to 31 years. In exposed to radiation areas were upper respiratory tract tumors (3 cases), thyroid tumors (11 cases), salivary gland tumors (2 cases), soft tissues tumors of anterior chest wall (2 cases), skin tumors (2 cases) – total 20 from 94 malignancies (21.3 %). The relative risk of postmetachronous malignancies calculated only for patients of the 1st and the 2nd group (1789 pts, 18,949 pts/years of observation) due to short followup of patients receiving chemoradiotherapy with reduced total focal doses (the 3rd group). In case of radio- and radiochemotherapy with 40 Gy total focal dose the relative risk of postmetachronous malignancies was 2.84 (95 % confidence interval (CI) 1.85–2.98); for women – 3.01 (95 % CI 1.9–3.12), for men – 2.45 (95 % CI 1.23–2.91). In women the relative risk of postmetachrinous malignancies was 3.22
(95 % CI 2.11–4.75) after independent RT and 2.89 (95 % CI 1.48–3.02) after radiochemotherapy; in man – 2.51 (95 % CI 0.9–4.63) and 2.44 (95 % CI 1.04–2.96), respectively. The relative risk common tumors: breast cancer – 4.01 (95 % CI 2.46–5.98); stomach cancer in women – 7.95 (95 % CI 3.2–14.4), stomach cancer in man – 4.03 (95 % CI 1.0–9.0); thyroid cancer in women – 7.81 (95 % CI 3.47–13.9).
Conclusion. Patients with HL are at higher risk of developing malignant neoplasms, compared with general population. They require medical follow-up in order to identify not only HL relapse, but postmetahronous malignancies.

Extranodal NK/T-cell lymphoma – a rare lymphoproliferative disease characterized predominantly by extranodal localization, aggressive course and low efficiency of conventional chemotherapy. Clinical presentation is diverse and depends on tumor lesion localization. In this article, a literature review and case report of patient with generalized extranodal NK/T-cell lymphoma with bone marrow involvement, unusual intracranial tumor localization, tumor leukemization and central nervous system-leukemia were presented. Adequate treatment strategy made it possible to achieve long-term complete remission in a patient with poor prognosis.

We conducted prospective study of the effectiveness of X-ray computed tomography (CT), magnetic resonance imaging (MRI) and MRI with diffusion-weighted imaging (MRI-DWI) with apparent diffusion coefficient (ADC) maps calculation for the differentiation of residual tumors and posttherapeutic masses in 40 adult patients with lymphoma. Whole body CT and MRI-DWI were performed before and after treatment.
The effectiveness of lesions size criterion for CT and MRI, visual and quantitative criteria for MRI-DWI were investigated. Residual lesions signal intensity on DWI images and ADC maps was compared with paraspinal muscles signal intensity. The accuracy of the overall tumor response estimation was 38 % for CT, 48 % for MRI, 68 % for MRI-DWI with visual assessment of DWI images, 93 % for MRI–DWI with visual assessment of ADC maps. CT density of the lymph node lesions before treatment and residual masses after treatment did not differ significantly – 40.4 ± 9.4 and 37.2 ± 10.5 Hounsfield units respectively (p = 0.08), whereas ADC (×10–3 mm2/s) increased significantly from 1.04 ± 0.40 to 2.01 ± 0.82 (p < 0.0001). ADC of postherapeutic masses was significantly higher than that of residual tumors – 2.32±0.62 and 1.04 ± 0.66 respectively (p < 0.0005). MRI-DWI with visual assessment of ADC maps is the most effective method for differentiation of residual tumors and posttherapeutic masses in patients with lymphoma after treatment. Usefulness of quantitative analysis of ADC values requires further investigation.

Objectives. The objective of this study was to evaluate epidemiology of febrile events (FE) and efficacy of antibiotic treatment in neutropenic patients with newly diagnosed acute myeloid leukemia (AML) with and without colonization of gut by extended-spectrum β-lactamaseproducing Enterobacteriacae (ESBL-E).
Materials and methods. The prospective study (2013–2015) included 66 patients with AML. These patients received 208 chemotherapy cycles within 6 month. Rectal swabs were obtained from all patients prior to antibiotic administration. ESBL-E were isolated on chromogenic ESBL selective medium CHROMagarТМESBL (CHROMagar, France) and confirmed by double disk synergy test.
Results. FE occurred in 193 (93 %) of chemotherapy cycles. The analysis was performed in 173 FE, including 68 – with colonization and 105 – without colonization with ESBL-E.
Epidemiology of FE was similar in patients colonized by ESBL-E and non-carriers group, except cases of bacteremia, caused by ESBL-E that occurred only in patients colonized by the same bacteria (7.5 %; p = 0.009). Patients colonized by ESBL-E and non-carriers had comparable efficacy of first-line non-carbapenem regimens (38 % vs 44 %), rate of carbapenem administration (62 % vs 55 %), efficacy of carbapenems alone (36 % vs 52 %) and in combination (64 % vs 41 %), duration of all antibiotics (14 days vs 13 days) and carbapenems (10 days vs 10 days). All cases of bacteremia caused by ESBL-E were successfully treated by carbapenems.
Conclusion. Colonization of gut with ESBL-E is a predictor of bacteremia caused by the same bacteria. There were no differences in the use of antibiotics in patients colonized by ESBL-E and non-carriers group.

Introduction. Bloodstream infections (BSI) are life-threatening illness for immunocompromised patients with hematological malignancies.
The aim of the study was to compare epidemiology, causative pathogens and outcome of hospital-acquired BSI and clarifying the role of herpes group of viruses in their development.
Materials and methods. During the period 1991–2013 438 bacterial strains obtained from 360 patients (pts) with hematological malignancies wеre studied. All blood cultures were incubated in the continuous monitoring system for 7 days before discard. The real-time PCR was used for human herpesviruses DNA detection: Herpes simplex viruses types 1 and 2 (HSV 1, 2), Cytomegalovirus (CMV), Epstein–Barr virus
(EBV) and Herpesvirus 6 (HHV-6). In this study 64 hematological cancer patients with infectious complications who fulfilled criteria of systemic inflammatory response syndrome with positive peripheral blood cultures were investigated. All pts received empirical anti-infectious therapy with subsequent correction based on the bacteriological, virological and mycological analyses.
Results and discussion. A total Gram-positive (G+) accounted for 69.2 % of BSI, Gram-negative (G–) for 30.8 %. Among G+ BSI Coagulase Negative Staphylococci and Staphylococcus aureus were the most frequent pathogens (58.8 %), among G– BSI Escherichia coli (13.0 %) was predominant. It is shown that the development of bacteremia were significantly more frequently occurs in the case of cytomegalovirus
and the Epstein–Barr virus detection.
Conclusion. Further epidemiological surveillance is warranted in order emerging resistant strains and related mortality. Reactivation of CMV and EBV is significantly associated with higher incidence of bacterial BSI.

Comparison of interpretation of acute lymphoblastic leukemia (ALL) flow cytometric diagnostics data was the aim of the study. Immunophenotyping data obtained from 10 patients with ALL were analysed separately in 26 laboratories from Russian Federation and Kazahstan. Results comparison showed four main type of discordance: B-lineage ALL diagnostics during heavy bone marrow regeneration, great variability of T-ALL interpretation, complexity of ambiguous lineage acute leukemia and, finally, very different report types, unique for each laboratory. All these problems are the serious obstacles for standardization of flow cytometric ALL diagnostics in multicenter setting. Continuation of similar QC rounds following by consecutive discussions with further development of consensus diagnostic algorithm could be the first step for standardization of ALL immunophenotyping in Russian Federation and CIS countries.

The literature review provides information on genetic diagnosis of Fanconi anemia: currently used methods of genetic analysis, spectrum and frequency of mutations, including in different populations, and order of molecular genetic methods are described. Problems of genetic diagnosis of Fanconi anemia in the world and in particular in the Russian Federation are also presented.

Multiple myeloma is a clonal B-cell malignancy characterized by proliferation of plasma cells that accumulate mainly in bone marrow and usually secrete monoclonal Ig and/or Ig light chains. The history of therapy development in this disease has more than 50 years. After ubiquitin-proteasome system of apoptosis become apparent bortezomib has been included in the mains therapy regimes. Simultaneously, the group of proteasome-inhibitor drugs is continually developing and opening more therapeutic options in refractory or relapse forms.