Relevance. Chronic myelogenous leukemia (CML) patients represent the heterogeneous group. Several studies in recent years were aimed to personalize treatment based on individual patients characteristics.
Aim of study. The aim of our study was to assess prognostic value of individual BCR-ABL decline rate in the first three months of CML therapy to predict optimal response.
Patients and methods. Fifty-four patients with chronic phase CML were included in the study. Forty-one patients started treatment with Imatinib 400 mg/day, 12 patients started with Nilotinib 600 mg/day and 1 patient started with Dasatinib 100 mg/day. BCR-ABL level was determined by International Scale at the moment of diagnosis and after 3, 6 and 12 months with ITK therapy. The ratio of BCR-ABL levels at 3 months to baseline for each patient, frequency of the achievement of the early molecular response at 3 months (10% by IS) and MMR at 12 months were assessed; in addition, we calculated ratio of BCR-ABL levels at 3 months to BCR-ABL levels at 1 month.
Twenty-six out of 34 patients (76.5%) with ratio of BCR-ABL levels at 3 months to baseline below than 0,1 achieved MMR at 12 months, while only 9 out of 20 patients (45%) with ratio more than 0,1 had optimal response (p = 0.02). Ratio of BCR-ABL levels at 3 months to BCR-ABL levels at 1 month showed much better results with the same (0.1) cut-off value – 5 out of 6 patients (83.3%) with ratio BCR-ABL levels at 3 months to BCR-ABL levels at 1 month below than 0.1, while only the 1 patient (16.7%) with ratio more than 0.1 achieved optimal response (p = 0.04), respectively. Application of early molecular response at 3 months (10% by IS) yielded worse discrimination results: 33 of 46 (71.7%) patients with BCR-ABL level ≤ 10% at 3 months, whereas 2 of 8 (25%) patients with BCR-ABL > 10% had MMR at 1 year (p = 0.02), respectively. Furthermore, application of our ratio cut-off value in patients with early molecular response (BCR-ABL level ≤ 10% at 3 months) allowed us to reveal additional 5 high-risk patients who have not reached MMR after 1 year of therapy.
Conclusion. Our study showed that individual BCR-ABL level decline rate estimated in the first three months of CML therapy from the baseline to the level measured at 3 months might be useful as an optimal predictor of outcome for CML patients (MMR after 1 year of treatment).

The relationship between the type of antitumor response to induction therapy in patients with multiple myeloma (MM) and/or monoclonal gammopathy of undetermined significance (MGUS) and the level of c-MYC gene expression at the moment of disease diagnosis is studied. The expression of c-MYC in bone marrow cells was evaluated by multiplex RT-PCR. The mean levels of c-MYC gene expression in CD138⁺ cells of patients with MM/MGOO are higher than in donors (p = 0.011). Relationship between the depth of antitumor response and level of C-myc expression in CD138⁺ cells is detected.

The aim of the study was to evaluate the quality of life (QoL) and symptoms in patients with myelofibrosis (MF). 93 patients with MF who participated in the multicenter observational study “Quality of life and symptoms in myelofibrosis: validation of the symptom assessment tool for patients with myelofibrosis” (2014–2015) were enrolled in the analysis. 62 patients received best available treatment (BAT) and 31 patients received ruxolitinib. All the patients filled out the SF-36, CSP-Myelofibrosis Module and Patient Global Impression of Change (PGIC) tools. In the real-world study in MF patients QoL was demonstrated to be significantly worse than in healthy controls; more than one third of patients had significant or severe QoL impairment. Patients treated with ruxolitinib had better QoL and lower symptom severity as compared to BAT patients. Decline of activity appeared to be the major symptom which significantly reduced QoL in MF patients. QoL and symptom data in MF patients during treatment in a real world setting may be a solid supplement to clinical variables and may contribute to better disease control in accordance with the principles of risk-adaptive therapy.

The aim of the study was to evaluate colonization with ESBL-E of gut and oropharynx in patients with acute myeloid leukemia (AML) and lymphoma before the start of the chemotherapy. 98 patients (33 with AML and 65 with lymphoma) were included in the prospective study (2013–2014). Newly diagnosed hematological malignancies were in 94 (96%) patients. Median age of patients with lymphoma and AML was 47 and 35 years. For the first two days after admission to the hospital swabs from gut and oropharynx in patients were taken. ESBL production was confirmed by phenotypic tests, the genes bla_CTX-M and bla_TEM - by real-time PCR. Upon admission to the hospital ESBL-E colonization of gut was detected in 27% patients (28% in AML and 24% in lymphoma) and only 4% patients had ESBL-E colonization of throat, p < 0,01. Total of 34 of ESBL-E isolates were detected (E. coli 52%, K. pneumonia 42%, Citrobacter spp. 6%). CTX-M type ESBL was detected in 76% of isolates, TEM type – in 53%, coexistence of TEM and CTX-M – in 44%. Independent risk factors for ESBL-E colonization in patients with lymphoma were transfer from another hospital (OR 4,2; p = 0,01) and age from 50 years and older (OR 3,0; p = 0.05); in patients with AML – place of living outside of Moscow (OR 7,6; p = 0,04). Multivariate analysis showed the same risk factors. Our results make doubtful the prescription of fluoroquinolones for prophylaxis without prior screening. Prophylaxis with fluoroquinolones may be optimal only for patients without ESBL-E colonization.

The role of regulatory T-cells (T-reg) in the development of autoimmune complications after allogeneic bone marrow transplantation (allo-BMT) is to control the augmented response of donor effector cells. The main mechanisms of immune regulation are the secretion of cytokines, induction of metabolic disturbances in target cells, modification of dendritic cells, direct cytolysis of T effector cells. One of the regulatory mechanisms that use T-reg is a direct cytolysis, the major mediator is granzyme B. Granzyme B plays a key role in maintaining the immune homeostasis. Preliminary data from a qualitative and quantitative assessment of granzyme B-positive T-reg in 30 patients after allo-BMT endorse this role.

The aim of the study was to analyze clinical significance of herpes viruses DNA monitoring in bronchoalveolar lavage fluid (BALF) of patients with hematological malignancies and pneumonia during or after chemotherapy. Samples of 171 enrolled patients were tested for human cytomegalovirus (CMV) DNA, herpes simplex virus I and II types (HSV) DNA, Epstein-Barr virus (EBV) DNA and human herpes virus 6 type (HV6) DNA. Viral load in BALF was also measured. Factors potentially increasing the rate of reactivation of endogenous herpes viruses have been evaluated. Among them: age of 50 years and over, laboratory sights of immunodeficiency like neutropenia and/or hypogammaglobulinemia, severity of condition/need for admission to ICU. Results. HSV DNA proved the highest detection in BALF frequency. Statistically significant difference for HSV DNA detection rate was obtained (Chi-sq. = 7.65; p = 0.022) for patients with all evaluated factors. HSV 1 and 2 of types were shown to be the most frequently identified viruses. In majority of samples HSV viral load in BALF lies within high values such as 30% and above of ICU patient’s BALF samples. In the vast majority of positive samples of DNA the concentration of this virus was in the area of high values (up to 10⁷ copies/ml).
Age over 50 years, and also such laboratory signs of immunodeficiency as neutropenia and/or hypogammaglobulinemia had no significant effect on the incidence rate of the development of herpes virus-associated pneumonia. There are no data evaluating the severity of virus-associated pneumonia against the background of immunodeficiency. There is no data describing the severity of the course of herpes virus associated pneumonia in patients with hematological malignancies.

Accumulation of excess iron in the organs and tissues significantly increases morbidity and mortality rates in patients with transfusion-dependent forms of anemia. The purpose of the study were to assess the degree of iron overload of the liver, myocardium, pancreas and pituitary gland in children of various ages with transfusion-dependent major form of β-thalassemia and to analyze the adequacy of ongoing chelation and transfusion therapy. 21 patient with β-thalassemia were included in the study. Prior to the study 6 children did not receive chelation therapy. In all children the serum concentrations of ferritin and soluble transferring receptor (rTFR) were examined and T2* mode MRI investigations of pituitary, myocardium, liver and pancreas were performed. For the assessment of the degree of liver fibrosis and hemosiderosis there was performed liver biopsy. There was correlation between the iron content in the myocardium and erythropoiesis activity (r = 0.230). There was revealed the high inverse correlation between the concentration of iron in the liver and the accumulation of iron in the pancreas (r = -0.585), an inverse correlation between the accumulation of iron in the pituitary gland and pancreas (r = -0.430). The most of the children (n = 12) had iron overload as of the pancreas, as well of the pituitary, which corresponds to a T2*MR signal time shortening, 8 patients had iron overload of the pituitary in allowable iron content in the pancreas, and only the one child had iron deposition neither in the pituitary nor in pancreas. Thus, the pituitary gland accumulates excess iron significantly earlier than the pancreas, but before the onset of puberty, the pituitary gland, these changes of the pituitary do not have clinical manifestations. Accumulation of excess iron in the body is accompanied by a damage of the pituitary gland with following involvement of the pancreatic gland and myocardium. The regression analysis revealed a non-linear relationship between the concentration of serum ferritin and iron accumulation in the pituitary gland. These results don’t allow to consider the serum ferritin concentrations as a marker of the accumulation of iron in the pituitary gland.

Von Willebrand disease (vWD) is a heritable disease, which can be inherited by either autosomal dominant or autosomal recessive, caused by qualitative or quantitative deficiency of von Willebrand factor (vWF) and characterized by excessive bleeding. In Russia 2013 was announced as a year of von Willebrand disease. For elaboration of guidelines on improvement of specific care of children and adults suffering from vWD we carried out a retrospective study. Information regarding the patients registered by regional haematologists with diagnosis of vWD (ICH-10 code D 68.0) was collected. There were examined 86 patients suspected for vWD. The diagnosis was confirmed in 52 (61.6%) patients. This data supported opinion that present status of laboratory service in the Russian regions is not satisfactory for detection of coagulation disorders and final diagnosis justification. Factor VIII concentrate is relatively novel but rare for Russian practice therapeutic approach for bleeding treatment in the patients with vWD. According to the results of the study it was effective in all the cases.

Vitamin K plays a special role in the blood clotting system, as this vitamin is essential for the synthesis of coagulation factors (II, VII, IX, X, proteins C and S) in the liver. Vitamin K in newborns matures to a certain time and the level of vitamin K dependent factors to the age of 6 months should achieve the standards of an adult human. The most frequent complications of abnormalities in the synthesis of vitamin K-dependent factors are hemorrhagic syndromes. There has recently been an increase in cases of hemorrhagic conditions not only in newborns but also in children of early age due to the lack of prevention of vitamin K deficiency at birth. The article analyzes the observation of hemorrhagic conditions developed after the neonatal period in patients with various chronic diseases.