Introduction.  The acute lymphoblastic leukemia (ALL) therapy programs developed by the BFM group (Berlin-Frankfurt-Munster) are used to treat lymphoblastic lymphomas from precursor cells (LBL) and are the most effective treatment methods in the world.

Aim. To assess   the treatment results of LBL in children according to the ALL IC-BFM 2002/2009 protocols in a clinical trial of a single center.

Materials and methods. From 2002 to 2022 a retrospective and prospective study included 70 patients aged 2 to 17 years with newly diagnosed LBL, with a median age of 9.4 years. There were 1.5 times more male patients than female (43:27). LBL from T-cells progenitor (T-LBL) was diagnosed in 61 patients (87 %), and LBL from B-cells progenitor (B-LBL) — in 9 (13 %). 59 (84.3 %) patients were included in the intermediate risk (IR) group and 11 (15.7 %) patients — in high-risk (HR) group. There were no patients in the study cohort who met the criteria of the standard risk group (SR).

Results. The 10-year OS in the group of patients treated according to the ALL IC-BFM 2002/2009 protocols was 95.4 ± 2.6 %; the 10-year RFS — 91.9 ± 4.9 %; the 10-year EFS — 86.7 ± 5.6 %. When analyzing survival rates depending on the prognostic risk group, the 10-year OS for patients of the intermediate risk group was 96.6 ± 2.6 %, and for patients of the high-risk group — 90.9 ± 8.7 %.

Conclusions. The obtained results confirm the high effectiveness of  the ALL IC-BFM 2002/2009 protocols in the treatment of LBL.

Introduction.  Plasmapheresis is a routine procedure in many blood  centers. The most common complications in plasma donors are vasovagal and citrate reactions. The effects of citrate exposure, repeated with different frequency over a long period  of time, have not been sufficiently studied.

Aim. to research the effect of the frequency of plasmapheresis on the concentration of ionic and protein composition in donor blood.

Materials and methods. The concentration of protein in blood serum was measured using a biochemical method. The concentration of ions was carried out using an automatic blood  electrolyte analyzer. Plasma donors were divided into groups depending on gender and frequency of plasmapheresis (1–5 times a year; 6–10 times a year; 11–26 times a year). The total number of donors involved in the study was 274 (155 men and 119 women).

Results. A significant decrease in the concentration of bound calcium was revealed in the group of female donors who donated plasma 11–26 times compared to the group who donated plasma 1–5 times a year. A significant decrease in the concentration of iron ions was revealed in the group of female donors who donated plasma 11–26 times compared to the group who donated plasma 1–5 times a year. Statistically significant differences in the concentrations of total protein were revealed between all three groups of male donors, and between groups of female donors who donated plasma 1–5 times and 6–10 times a year; 1–5 times and 11–26 times a year.

Conclusion.  The use of data on changes in the parameters of ion and protein metabolism in donors of different sexes and different donation frequencies reduces the risk of developing a citrate reaction due to the selective selection of plasma donors.

Introduction.  The high frequency of medical withdrawals from donatio ns due to a decrease in hemoglobin levels and the negative impact on the motivation and return to donation of temporarily withdrawn individuals dictates the need to study the effect of exfusion of blood and its components on iron metabolism.

Aim:  to evaluate the iron metabolism indicators  in regular donors of platelets and plasma.

Materials and methods. 99 platelet donors and 90 blood plasma donors who regularly participated in donations were examined. The comparison group consisted of 158 healthy individuals who had not previously donated blood and its components. Hemoglobin concentration, hematocrit, erythrocyte count and erythrocyte indices were assessed. The concentration levels of ferritin, iron, transferrin in blood serum as well as  the values of total and unsaturated iron-binding capacity of se rum were studied, and the saturation coefficient of transferrin was calculated.

Results. In men who donated platelets, a statistically significant decrease in the red blood cells count (p = 0.001), hematocrit (p = 0.014) and ferritin concentration (p < 0.001) were found relative to those in the comparison group: 4.8×10¹²/l, 42.0 %, 28.9 ng/ml versus 5.0×10¹²/l, 44.0 %, 74.2 ng/ml respectively. In regular  donors a significant increase in the medium of hemoglobin of red blood cells was noted: 30.8 pg (p =  0.008) and 31.4 pg (p <  0.001) in men and 30.2 pg (p =  0.030) and 31.0 pg (p < 0.001) in women with platelet and plasma exfusions respectively compared to 29.4 pg in men and 29.6 pg in women who participated in donation for the first time. In plasma donors of both sexes, the values of hemoglobin and its average concentration in the erythrocyte were significantly higher than in first-time donors: 154.0 g/l (p = 0.008), 35.7 g/dl (p < 0.001) and 138.0 g/l (p < 0.001), 35.2 g/dl (p < 0.001) versus 146.0 g/l, 33.6 g/dl and 129.0 g/l, 33.8 g/dl in men and women, respectively.

Conclusion.  To prevent the development of iron deficiency in donors of blood components it is necessary to monitor the con-centration of serum ferritin and correct its reduced values by changing the donation regimen or prescribing iron-containing drugs. When selecting donors, attention should be paid to the  frequency of apheresis, especially in men who regularly donate platelets.

Introduction.  The physiological task of the blood coagulation system  during childbirth is to prevent excessive blood loss.

Aim:  to evaluate the quantitative and qualitative characteristics in the system P-selectin-von Willebrand factor (vWF)-metallo-protease ADAMTS13 during natural and uncomplicated  childbirth in women with a physiological pregnancy.

Materials and methods. The study included 40 pregnant women without somatic pathology, with a physiologically progressing pregnancy that ended with physiological birth (general  group). Of these, 16 women had their first birth (group 1), 18 had their second birth (group 2) and 6 had their third birth (group 3). Venous blood sampling was performed at three points throughout the study: 1st point — the onset of labor, 2nd point  — 2 hours after childbirth and 3rd point — 24 hours after child -birth. The control group consisted of 25 healthy non-pregnant women. The blood samples were examined for: vWF antigen (vWF:Ag), binding ability of vWF to platelet receptor GPIb (vWF:GPIb), binding ability of vWF to collagen type I (vWF:CBAI) and type III (vWF:CBAIII), antigen and activity ADAMTS13 (ADAMTS13:Ag and ADAMTS13:AC), antibodies to ADAMTS13 (ADAMTS13:AB) and P-selectin.

Results. The medians of the studied vWF characteristics in the genera l group were statistically significantly higher compared to the control group at all study points, with the exception of the medians of the vWF:GPIb/vWF:Ag ratio, which in the general group were statistically significantly lower at all study points when compared with the control group. The median values of vWF:GPIb and vWF:Ag in women in the second group were statistically significantly higher at all points of the study, compared with similar indicators in both the first and third groups. The median values of ADAMTS13:AC and ADAMTS13:AB in the overall group were statistically significantly higher when  compared with the control group at all study points. Median ADAMTS13:Ag and ADAMTS13:AC/ADAMTS13:Ag ratios were statistically significantly lower at all study points. Individual P-selectin values varied widely from 212.1 to 1398 ng/ml, but the medians of this indicator were statistically significantly higher in all study groups and at all study points compared to the control group.

Conclusion.  The increase in platelet-binding and collagen-binding abilities of vWF suggests that these adhesive properties of the hemostatic protein play an important role in preventing excessive bleeding during labor. Taking into account the decrease in the vWF:GPIb/vWF:Ag ratio compared to the control group, the adhesion of vWF to collagen is more important under these conditions. Despite the fact that ADAMTS13 concentration does not decrease, an actual twofold increase in its activity provides a sufficient antithrombotic effect. The interaction between vWF and ADAMTS13 under conditions of physiological labor does not lead to excessive formation and accumulation of high-affinity antibodies to ADAMTS13.

Introduction.  Myeloproliferative neoplasms (MPNs) are a rare but significant cause of strokes.

Aim. To describe risk factors for stroke development, as well as  prevention strategies based on available literature data on patients with MPN.

Main findings.  Online databases (PubMed, E-Library) were searched for studies on cerebral thrombotic complications in the setting of myeloproliferative disorders. Induction of specific cytoreductive treatment with  low doses of acetylsalicylic ac id should be considered as a mandatory component of secondary prevention of noncardioembolic stroke.

Introduction. Patients with hematological malignancies are one of the most vulnerable groups to infectious diseases. Immune system dysfunction caused by the disease and/or its therapy negatively affects infection disease duration and severity, leads to an increased risk of death, and reduces the efficacy of vaccination. However, the precise compensatory mechanisms that allow patients with deficient cellular or humoral responses to fight viral infections have not been sufficiently studied.

Aim: To systematize the knowledge of immune response formation in patients with hematological malignancies with humoral or cellular dysfunction.

Main findings.  In this review the features of the T-cell immune response in patients with hematological malignancies is explored, followed by a discussion on how the diversity of the T-cell receptor repertoire may be important in effective virus elimination and protection against SARS-CoV-2 virus infection.

Introduction.  The main manifestation of hereditary coagulopathies is bleeding, but patients with these diseases are not protected from thrombotic complications.

Aim: to evaluate the frequency and nature of thrombotic complications in various bleeding disorders.

Main findings.  In some bleeding disorders, such as von Willebrand disease, thromboses are extremely rare, while in others, such as dysfibrinogenemias, thrombosis is one of the key manifestations. For hemophilia A, there has recently been a significant expansion of treatment options. The use of some new drugs has already been associated with thrombotic complications in the clinical trial phase.

Introduction. A characteristic symptom of hemophilia is joint hemorrhage (hemarthroses).

Aim: to evaluate the possibilities of radiosynovectomy in hemophilic arthropathies.

Main findings. Radiosynovectomy (RSE) or radiosynoviorthesis (RSO) is a technique that achieves a long-lasting effect in chronic synovitis and hemophilic arthropathy. It consists of intra-articular injection of radiopharmaceuticals (RFLP) in the form of colloidal solutions, macroaggregates or microspheres containing radioactive isotopes, which are rapidly phagocytized by the covering cells of the synovial membrane. After such capture, the synovial sheath is subjected to intensive “internal” irradiation, which results in superficial fibrosis of synovial tissue and persistent suppression of joint inflammation.

Introduction. The development of hemorrhagic syndrome at the onset of the disease in patients with acute promyelocytic leukemia (APL) is one of the main causes of therapy failures and the most common cause of death in the early stages of remission induction. The risk of severe hemorrhagic complications increases in patients with the microgranular APL variant, often accompanied with hyperleukocytosis.

Aim — to present a clinical case of successful diagnosis and treatment of primary APL with initial hyperleukocytosis in a patient with massive intracranial hemorrhage and differentiated syndrome.

Main findings. A clinical observation of the diagnosis and treatment of APL in a 13-year-old patient with initial hyperleukocytosis, massive intracranial hemorrhage and differentiation syndrome is presented. Data on the variable manifestations of hemorrhagic syndrome in patients with APL and its effect on survival outcomes are also given. Diagnostic criteria and approaches in the treatment of differentiation syndrome are presented on the example of a clinical observation, when early clinical diagnosis and timely initiation of special antitumor treatment in the intensive care unit made it possible to achieve clinical, hematological and molecular remission, avoiding the fatal consequences of massive intracranial hemorrhage.

Introduction. Thrombophilia is a common cause of neurological complications.

Aim. to present a clinical observation of the development of ischemic stroke in a patient with hereditary thrombophilia.

Main findings. A case is presented of a man, aged 30, who developed an ischemic stroke in the basin of the left medial artery. A diagnostic study revealed that the development of the ischemic stroke was facilitated by thrombophilia caused by a combination of polymorphic variants of the fibrinogen genes (G-455A(G-467A)) and methylenetetrahydrofolate reductase (C677T). Thus, hereditary thrombophilia was a risk factor for the development of ischemic stroke in the patient at a young age. Therefore, in the absence of traditional risk factors for the development of ischemic stroke, a genetic analysis for thrombophilia is necessary.

Introduction. Renal artery stenosis is a common pathology associated mainly with atherosclerosis of the renal arteries. Essential thrombocythemia (ET) is a rare cause of renal artery stenosis.

Aim: to describe a clinical observation of a patient with ET, complicated by renal artery stenosis of a solitary functioning kidney, successfully corrected endovascularly.

Main findings. A 32-year-old male with ET, complicated by occlusion of the abdominal aorta, left renal artery, with shrinkage of the left kidney, subtotal stenosis of the right renal artery and chronic kidney disease stage 3A, as well as renovascular hypertension, underwent an organ-preserving intervention — endovascular correction of renal artery stenosis (ECRAS). As a result of ECRAS, the prescription of anticoagulant-antiplatelet therapy and specific therapy for ET with interferon, positive effects were observed in the form of improved kidney function, achievement of target blood pressure level, as well as in relation to basic hematological parameters.