Treatment of acute lymphoblastic leukemia (ALL) in children during the last 50 years has changed significantly, which has increased the survival of patients from 10–15 % in the early 60s to 80–85 % by the mid-2000s. Such results have been achieved through the development of new polychemotherapy regimens, the introduction of neuroleukemia prophylaxis, the strengthening of standard chemotherapy by increasing the dose and / or frequency of chemotherapeutic drugs administration, and the definition of criteria for patient stratification into prognostic risks groups and the development of principles of risk-adopted therapy.

However, inspite of the overall success of pediatric acute lymphoblastic leukemia therapy, some variants of acute lymphoblastic leukemia associated with poor prognosis, especially acute lymphoblastic leukemia with BCR-ABL1 and MLL rearrangements. Besides the prolonged persistence of minimal residual disease is also an unfavorable prognostic factor requiring therapy intensification.

In the current issue we present the main steps in the evolution of programmed chemotherapy of children with acute lymphoblastic leukemia. Great attention was paid for modern risk-stratifying criteria with an emphasis on minimal residual disease.

Background. Bendamustine is one of the main drugs included in the arsenal of cytostatic agents that are used in oncohematology. In Russia, the drug was registered at the end of 2010 under the commercial name Ribomustin (company Astellas). At the end of 2018, two national generics of bendamustine appeared on the Russian market – Rozustin® (Rafarma) and Kovada (Biocad).

Materials and methods. In 2019 the department of hemoblastosis chemotherapy of the N. N. Blokhin National Medical Research Center of Oncology received the drug bendamustin (Rozustin®) (Rafarma, Russia). This article presents the first experience of using Rozustin® in patients with various lymphoproliferative diseases. Most often, the drug was used for relapsed or refractory classical Hodgkin’s lymphoma.

Results and conclusion. The high effectiveness, acceptable toxicity profile compared to alternative “salvage” regimens, ease of implementation, and the possibility of a full collection of hematopoietic stem cells followed by high-dose chemotherapy and autologous stem cell transplant, confirm the good potential of the combined program with the inclusion of the drug bendamustine (Rozustin®).

Background. The prognosis of patients with multiple myeloma (MM) is significantly different depending on the biological characteristics of the tumor substrate, the microenvironment of the bone marrow, as well as factors associated with the patient’s body. Therefore, the search for new reliable and easily identifiable prognostic markers is relevant for the effective management of patients with this disease.

The objective of the study was to assess the prognostic value of the study of serum free light chains (FLC) of immunoglobulins κ and λ and their ratio κ / λ FLC in the blood serum of patients with newly diagnosed MM in real clinical practice.

Materials and methods. 369 patients with first diagnosed MM (134 men and 235 women) were examined who were hospitalized in the hematology department of the City Clinical Hospital No. 2 Novosibirsk in the period since January 2012 to December 2017. The median age of the patients was 67 (32–82) years. All patients received induction courses of chemotherapy based on bortezomib. The control group consisted of 56 conditionally healthy individuals: 34 women (60.7 %) and 22 (39.3 %) men with a median age of 62 (40–68) years. The concentration of FLC-κ and FLC-λ (mg / L) in blood serum was determined by immunoturbidimetric method on a Hitachi 911 automated biochemical analyzer using the Freelite Human Lambda and Freelite Human Kappa reagent kits (Binding Site, Great Britain).

Results. It was found that in patients with MM, the concentration of serum FLC-κ or FLC-λ was statistically significantly higher compared to the control group and varied depending on the type of MM (p <0.001). The diagnostic sensitivity of the quantitative determination of FLC and their ratio for MM was 98.64 %, compared with 94.04 % in a standard immunochemical study. The values of the ratio κ / λ FLC <0.04 or> 65, as well as the concentration of FLC-κ and FLC-λ are higher than the median obtained in the whole group (FLC-κ ≥702 mg / L and FLC-λ ≥493.2 mg / L), correlate with known factors of poor prognosis for MM (with a high concentration of β2‑microglobulin (>3.5 mg / L) (r = 0.461; p <0.001), plasma cell bone marrow infiltration >60 % (r = 0.420; p <0.001), renal failure (creatinine >177 μmol / L) (r = 0.380; p = 0.002), and also with high lactate dehydrogenase activity (>450 U / L) (r = 0.520; p <0.001) and is associated with poor outcomes. The median overall survival in the group of patients with κ / λ FLC <0.04 or >65 was 49 months compared to 76 months in the group with κ / λ FLC 0.04–65 (log-rank p = 0.012).

Conclusion. The determination of free FLC in the blood serum of patients with MM can be used to assess the prognosis of their survival. The value of the κ / λ FLC ratio <0.04 or >65 allows us to divide patients with MM into risk groups with significantly different outcomes and can be used to identify patients at high risk who need more aggressive therapy and more detailed monitoring of the response.

Background. An increase in the number of patients with multiple myeloma (MM) necessitates the creation of reliable tools for assessing their somatic status (comorbidity) in order to personalize the optimal treatment regimen that helps to minimize its toxicity, improves survival and patients quality of life.

The objective of this study was to modify the MM comorbidity index (MCI) by adding an additional variable reflecting the biological properties of the tumor, and to determine the informativeness of the new scale – a modified MM comorbidity index (M-MCI), to predict the outcome and select personalized therapy in patients with MM in real clinical practice.

Materials and methods. From January 2012 to December 2017 the study included 369 patients with newly diagnosed MM (134 men and 235 women) who were hospitalized in the hematology department of the City Clinical Hospital No. 2, Novosibirsk. The median age of the patients was 67 (32–82) years. The prognostic value of concomitant diseases and individual prognostic factors in relation to the overall survival of patients with MM was evaluated.

Results. Cox multivariate analysis showed that the most significant predictors of reduced overall survival of patients with MM are impaired renal function (glomerular filtration rate <30 ml / min / 1.73 m2 (according to the CKD-EPI formula), general condition according to the Karnowski scale ≤70 %, chronic obstructive pulmonary disease with moderate (50 % ≤ forced expiratory volume in 1 second <80 %) and severe (30 % ≤ forced expiratory volume in 1 second <50 %) severity of bronchial obstruction and the ratio κ / λ free light chains <0.04 or >65. These factors were combined into a weighted 5‑point scale M-MCI. A comparative analysis of survival depending on the value of the M-MCI allowed us to distribute patients with MM into groups of high (M-MCI 3–4 points) and standard (M-MCI 0–2 points) risk with significantly different indicators of overall survival (median overall survival amounted to 15.5 months in the high and 60 months in the standard risk group; χ2 = 58, p <0.016) and confirm the prognostic value of M-MCI in relation to the outcome of MM.

Conclusion. In terms of its prognostic significance in predicting an adverse outcome, the proposed M-MCI scale is superior to its prototype – the MCI. The median overall survival in the high-risk group according to the M-MCI was 15.5 months compared to 20 months according to the MCI; the median overall survival in the group the standard risk was 60 and 50 months, respectively (χ2 = 58 (M-MCI) versus χ2 = 42 (MCI); p <0.001). The advantages of M-MCI are also its more accurate assessment of the physical condition of patients with MM and its simple clinical applicability.

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) have an aggressive, life-threatening course. 5‑year survival rate is less than 20 %, which may be due to not timely diagnosis. PTCL-NOS can histologically and immunophenotypically mimic other T-cell lymphomas of the skin, including mycosis fungoides. In this connection, the correct diagnosis is most often established in the late stages of the disease. We present a clinical case of PTCL-NOS misdiagnosed as mycosis fungoides.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome associated with Castleman disease, monoclonal gammopathy of undetermined significance, symptomatic hemoblastosis or systemic autoimmune diseases. Slow development, nonspecifity of most clinical manifestations and lack of awareness of physicians are the main reasons for the late access to medical care and a long, sometimes many years, diagnostic search. Even after diagnosis, the use of modern methods of immunosuppressive, immunomodulating or antitumor therapy is significantly limited by the difficulties of taxonomic classification of the diagnosis, the lack of unified therapeutic approaches, and related unresolved issues of profiling and drug provision of patients. The article provides an overview of the basic information about the pathogenesis and clinical manifestations of POEMS syndrome. Using the example of a patient with Castleman’s disease, the difficulties of primary diagnosis of POEMS syndrome and the problems of interdisciplinary interaction of doctors of various specialties are demonstrated. The significance of endocrine disorders in the diagnosis and treatment planning is analyzed.

This review includes information about epidemiological data, development feature, phenotypes, principles of classification, diagnosis, and prognosis for drug-induced liver injury (DILI). Actual clinical recommendations regarding management of the DILI arising in the chemotherapy of cancer discussed. Drugs that can influence on individual pathogenetic mechanisms of growth and symptoms DILI are considered. Clinical studies for the prophylaxis and treatment of DILI associated with anticancer chemotherapy analyzed in detail. The prevention and treatment regimens for DILI in patients receiving chemotherapy and immunotherapy various localizations of cancer given both in research and in practical recommendations.

Anemia in patients with malignant neoplasms affects the quality of life of the patient and sometimes limits the timely implementation of antitumor treatment. In the pathogenesis of anemia of the malignant neoplasms the largest role play infiltration of the bone marrow by tumor cells, suppression of hematopoiesis by inflammation cytokines, development of functional iron deficiency, reduction of sensitivity of receptors to erythropoietin or its synthesis. The doctor can prescribe effective pathogenetic therapy after evaluating the mechanisms of anemia development in this category of patients. In the article are described in detail the methods of pathogenetic correction of anemic syndrome using parenteral iron preparations, recombinant erythropoietin, indications for their appointment, effectiveness in patients with cancer, as well as possible side effects and complications of therapy. The mechanisms of action, pharmacokinetics, and features of the use of different erythropoietin adents are described. It is shown the effectiveness of erythropoietin preparations in patients with lymphoproliferative disorders based on the results of our own study. A positive response was observed in 77.3 % of patients with non-Hodgkin’s lymphomas, in 61.8 % – with multiple myeloma and 60.9 % with chronic lymphocytic leukemia. It is presented the prognostic factors for the response to erythropoietin therapy and showed own datum in patients with myelodysplastic syndrome (in case of the serum erythropoietin <500 mMU / ml a positive response was found in 35.6 %, with higher level – no response) and with lymphoproliferative disorders (in case of erythropoietin was <130 mMU / ml, the positive response was 80 %, at 130–499 mMU / ml – 63.6 %, and at ≥500 mMU / ml – 25 %). In the article are described the principles of anemia correction using red blood cells transfusions and features of their use in patients with cancer. Special attention is paid to the study of blood saturation as one of the indicators that allow us to assess the adequacy of the gas transport function of blood during red blood cells transfusions. The algorithm for correcting anemia in malignant neoplasms using red blood cells transfusions and erythropoietin agents are presented. It is shown low blood saturation (<60 %) in 32 % hematological malignancie’s patients with a hemoglobin 8.0 g / dL. This datum suggests presence of tissue hypoxia and gives approval to expand the threshold for red blood cells transfusions

Finding opportunities to improve treatment outcomes of cancer patients remains a difficult and unresolved problem. Modern anticancer treatment due to the intensity and molecular biological orientation allows achieving higher efficiency and theoretically reducing the complications frequency. At the same time, the “increase in efficiency” in the modern oncology really exists, but a “decrease in the incidence of complications” is far from its solution. In many ways, the problems of diagnosis, treatment and monitoring of complications are associated with the impact on complex physiological processes in the body of an oncological patient. Timely implementation of modern and adequate programs for the prevention and treatment of these complications defines the concept of “supportive therapy”, which provides at least half of the effectiveness of anticancer treatment.

The Multinational Association of Supportive Care in Cancer (MASCC) was formed in 1990. The main tasks of the association were the creation of supportive care system, its popularization and accumulation of scientific data. The MASCC was used not only oncologists experience, but also of specialists working in almost all areas of medicine. Supportive therapy provides prevention and treatment of complications from the moment of malignant disease develops, at all stages of anticancer treatment, during the rehabilitation period, and in patients in the terminal phase.

An important stage in the development of maintenance care in Russia was the holding of annual conferences in Moscow with the support of MASCC. Russia is included in the European MASCC group and in working group on supportive therapy and palliative care of the Chemotherapists Society (ESMO). The Russian Society of Supportive care in Oncology (RASSC) was organized In Russia on June 1, 2017. In recent years, the main directions of supportive care have been developed in our country. Today, supportive therapy is an obligatory component of anticancer programs, which allows the patient to cope with severe but potentially reversible disorders of vital organs at all stages of treatment. This is a real way to increase the treatment efficacy and improve the quality of life of cancer patients.