Background. Primary immune thrombocytopenia (ITP) is an orphan disease characterized by decreased platelet count in the peripheral blood which in some cases can cause bleeding of varying severity. Currently, the use of thrombopoietin receptor agonists (TPO-RAs) is recommended as the second line therapy for ITP as it allows to achieve high platelet response (PR), including complete, in 73 % of cases of chronic ITP and in 87 % of cases of newly diagnosed disease. The mechanism of action differs for different TPO-RAs. Given this fact, in cases of resistance or intolerance to therapy with one TPO-RA, attempts are made to switch to another. The effectiveness of this approach for overcoming ITP resistance varies from 50 to 93 % according to various publications.

   Aim. To assess the ability to achieve and maintain PR by switching from one TPO-RA to another in cases of resistance to the previous TPO-RA used in the second or subsequent lines of therapy.

   Materials and methods. The analysis included 59 patients who were resistant (in 2 cases intolerance was also noted) to TPO-RA therapy (received after standard therapy) who were prescribed TPO-RA treatment with a different mechanism of action: switch from romiplostim to eltrombopag (25 patients) or vice versa (34 patients). Both groups were comparable in terms of demographic characteristics and median platelet level at the time of TPO-RA switching.

   Results. PR was obtained in 76 % of cases, including complete response in 54 %, as a result of switching from one TPO-RA to another in 59 patients. Among 34 patients switched from eltrombopag to romiplostim, PR was achieved in 31 (91 %) patients, including complete response in 22 (65 %). Romiplostim was switched to eltrombopag in 25 patients, PR was achieved in 14 (56 %) with complete response in 10 (40 %).

   Conclusion. The study showed that PR can be achieved and maintained through switching from one TPO-RA to an alternative.

   Background. Early (stage I–II) classical Hodgkin lymphoma is a highly treatable malignant neoplasm. For many years, the standard treatment of early Hodgkin lymphoma has been combination therapy: polychemotherapy with subsequent consolidating radiation therapy.

   Aim. To retrospectively evaluate the results of treatment of stage I–II classical Hodgkin lymphoma in a favorable prognosis group using monotherapy and combination therapy (chemotherapy followed by consolidating radiotherapy).

   Materials and methods. The study included 58 patients aged 19–81 years (median age 36 years) diagnosed with stage I–II classical Hodgkin lymphoma with favorable prognosis; 23 (39.7 %) patients were men, 35 (60.3 %) were women. Depending on the treatment received, the patients were divided into two groups: 40 (69 %) patients received ABVD polychemotherapy, and 18 (31 %) patients received polychemotherapy followed by consolidating radiation therapy to the initial lesion zones with standard fractionation regimen (total dose 30 Gy). The Kaplan–Mayer method was used to evaluate overall, progression-free, recurrence-free, and event-free survival. Odds ratios and their 95 % confidence intervals were also calculated. The statistical significance of differences in survival was assessed using the log-rank test. The level of statistical significance was 0.05. Statistical data processing was carried out using the IBM SPSS Statistics 27 software and Microsoft Excel.

   Results. According to the results of our study, while in the total patient group a clear numerical advantage in survival (overall, progression-free, relapse-free and event-free survival) was observed in the combination therapy group compared to chemotherapy group (5.5, 13.2, 9.2 and 23.9 %, respectively), no preferred tactics were identified in the total patient group. However, considering a subgroup of patients with mediastinum involved in the tumor process, a statistically significant advantage of combined treatment over single-mode chemotherapy was determined for 2-year event-free survival (92.9 ± 6.9 % vs. 62.5 ± 9.9 %, respectively, p = 0.046). The risk of an adverse event is reduced by >80 % (odds ratio 0.197; 95 % confidence interval 0.036–0.977) when using consolidating radiation therapy after chemotherapy.

   Aim. To study the recurrence of stage I–II breast cancer after subcutaneous/skin-sparing mastectomies with reconstruction with or without radiation therapy.

   Materials and methods. From 2013 to 2022, 984 patients diagnosed with breast cancer underwent 1020 subcutaneous/skin-sparing mastectomies with reconstruction at the P.A. Herzen Moscow State Medical Institute. Histological types are presented: cancer in situ – 7, invasive cancer without signs of specificity – 818, invasive lobular cancer – 105, combined cancer – 40, rare forms – 50. Subcutaneous mastectomy with reconstruction was performed in 617 (60.5 %), skin-sparing mastectomy in 403 (39.5 %) cases. Reconstruction with own tissues was performed in 5.7 %, expanders/implants in 94.3 % of patients. Patients with diagnosed mutations in the genes ВRCА1, 2, СНЕК2 amounted to 208 (21.1 %), while among patients with primary multiple synchronous cancer, the percentage with mutations was 25 %.

   Results. In the studied group of patients, recurrence of breast cancer was diagnosed in 40 (4.1 ± 0.1 %) cases, distant metastases – in 52 (5.3 ± 0.1 %) cases. The minimum time to relapse was 36 months, and the maximum was 108 months. In a single-factor analysis of the stage of the disease: at stage I, relapse was diagnosed in 25 (5.8 %) cases, at stage IIA (T2N0M0) – in 5 (2.0 %), at stage IIA (T1N1M0) – in 6 (4.2 %), at stage IIB T2N1M0 – in 4 (2.1 %) (p > 0.05). The recurrence in the group with radiation therapy was 2.5 ± 0.6 (odd ratio 0.98, 95 % confidential interval 1.52–3.48), without radiation therapy 5.1 % (odd ratio 2.13, 95 % confidential interval 0.92–5.18) regardless of the stage of breast cancer (t-criterion >2, p > 0.05). In our study, the recurrence of breast cancer at the edge of R1 was 6.8 ± 2.5 %, at R0 – 3 ± 0.6 % (p > 0.05). When analyzing age and risk of recurrence, we did not identify age dependence, up to 40 years the probability of recurrence is 4.3 ± 1 %, after 40 years – 3.3 ± 0.7 % (t-criterion 0.44, p > 0.05). The dependence of the degree of malignancy of the tumor node and the frequency of recurrence is as follows: G1 – 2. 3 ± 2.3 %, G2 – 3 ± 0.7 %, G3 – 3.9 ± 1 %, when compared G3 c G1 (t-criterion 0.52, p>0.05), when comparing G3 from G2 (t-criterion 0.49, p > 0.05). Recurrence of breast cancer with triple negative type was diagnosed in 2.5 ± 0.9 % relative to other molecular biological types (t-criterion 1.49, p > 0.05). We analyzed the dependence of relapse on the non-luminal and luminal HER2 positive types; the relapse was 5.4 ± 1.5 % (p > 0.05); the dependence on the level of Ki-67; at a level of 40 %, the probability of recurrence is 4 ± 0.8 %, at a level > 40 %, recurrence is 2 ± 0.8 % (t-criterion 1.77, p > 0.05). A statistically significant difference in our study was revealed, in addition to radiation therapy, during neoadjuvant polychemotherapy, so in the group with neoadjuvant polychemotherapy, the recurrence was 2 ± 0.8 %, without neoadjuvant polychemotherapy – 4.1 ± 0.7 % (t-criterion 2.16, р < 0.05).

   Conclusion. A link between breast cancer recurrence and morphological features (R1 resection margin, lymphovascular invasion, lymphatic tumor emboli, luminal and non-luminal HER2+ subtype, G3) and clinical characteristics (presence of mutations, location of a lesion in the gland, stages) was found. Further search for predictors of breast cancer recurrence after combination or integrated treatment is necessary.

   About 15 % of lung tumors are small cell lung cancer. Because of its extremely poor prognosis with a median overall survival of 10 months, and the long absence of new treatment options beyond the combination of platinum and etoposide (EP), the introduction of immunotherapy has been a significant development in the treatment of this disease. IMpower133 was the first phase III trial demonstrated a statistically significant increase in median overall survival in the atezolizumab group from 10.3 to 12.3 months. Our clinical case demonstrates the efficacy of an EP with atezolizumab. The patient got 6 courses of EP with atezolizumab and 29 courses of atezolizumab maintenance immunotherapy, indicating the efficacy and tolerability of this combination.

   Chronic lymphocytic leukemia (CLL) is a B-cell tumor consisting of small lymphocytes. It develops through a multistage process of a series of genomic events. Bruton’s tyrosine kinase (BTK) plays an important role in signal transduction through constantly active BCR pathway. It participates in all aspects of B cell development including proliferation, maturation, differentiation, and apoptosis. Therefore, it seems reasonable to modulate BTK using pharmaceutical agents with the goal to suppress tumor process. The effect of 1st generation BTK inhibitor ibrutinib on non-target kinases is significant and causes some adverse events which can limit its use in older patients with concomitant pathologies. The results of completed trails have convincingly shown safety advantage and similar effectiveness of highly selective 2nd generation BTK inhibitor acalabrutinib compared to ibrutinib in all subgroups of patients with CLL. Considering the necessity of long term BTK inhibitor therapy (until progression or unacceptable progression), long-term manageable safety profile of acalabrutinib is important. The article discusses clinical pharmacology, effectiveness and safety of acalabrutinib therapy in the context of clinical trials. Analysis of medical histories of patients with CLL treated at the Clinical and Diagnostic Center of the Lapino Clinical Hospital, “Mother and Child” group of companies, in the last year was performed, and indications for treatment using 2nd generation BTK inhibitor were evaluated.

   Background. Currently, the main method of treatment of locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses is surgical treatment with subsequent radiation/chemoradiation therapy. However, the survival rate of patients has not changed significantly in the last 20 years. Head and neck squamous cell carcinoma is highly sensitive to chemotherapy and radiation which allows to perform treatment even in cases of locally advanced squamous cell carcinomas of the nasal cavity and paranasal sinuses. However, the question of prognostic factors of the effect of chemoradiation therapy remains open. Therefore, determination of these factors is an important problem.

   Aim. To evaluate the effectiveness of conservative treatment of locally advanced squamous cell carcinoma of the nasal cavity and paranasal
sinuses.

   Materials and methods. The retrospective analysis included 55 patients with squamous cell carcinoma of the nasal cavity and paranasal sinuses (stages III–IVA) who were under observation and receiving treatment at the N.N. Blokhin National Medical Research Center of Oncology between 2000 and 2020. In 50 (90.9 %) patients, treatment began with induction chemotherapy with subsequent radiation therapy per the radical program. In this patient group, treatment effectiveness was evaluated per the RECIST 1.1 (Response Evaluation Criteria In Solid Tumors) criteria.

   Results. Five-year overall and progression free survival rates were 49.5 and 22.5 %, respectively. Objective response rate after treatment was achieved in 65.5 % of cases: complete response was observed in 38.2 % of cases (n = 21); partial response in 27.3 % (n = 15); stable disease in 18.2 % of cases (n = 10); disease progression in 16.4 % (n = 9).

   Conclusion. According to the results of the study, conservative treatment is a promising approach in locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses. Patients with complete response to treatment have the highest odds of cure and improved long-term outcomes.

   The most common cause of total temporomandibular joint (TMJ) prosthesis is the terminal stage of degenerative dystrophic diseases. Patients with oncologic pathology usually undergo extended surgical interventions with TMJ exarticulation which in contrast to non-tumor pathology are characterized by resection of the whole ligamentous apparatus of the joint, the ramus of the mandible, and surrounding structures. Due to various causes, the use of traditional systems of open-type joint prosthesis (without fixation of the joint head in the glenoid fossa) frequently leads to loosening, luxation, and eventual dislocation of the joint head from the glenoid fossa. Currently, multiple different TMJ prosthetic systems have been proposed but only 2 are routinely used: TMJ Concepts (USA) and TMJ Biomet (USA). It should be noted that these systems are also open. We have studied the used constructions of total TMJ endoprostheses and haven’t found any that solve the problem of the condyloid component dislocation from the glenoid fossa after extended resections due to tumors of the TMJ. Moreover, analysis of the clinical outcomes after the use of such systems showed that even in cases of relatively limited resections of TMJ structures (11–30 %), joint prosthesis elements dislocation still occurs. Therefore, a promising solution for the problem of the joint head dislocation can be development of a closed total endoprosthesis with joint head fixation and preservation of joint mobility in all planes.

   Using clinical cases of patients observed at the Endoscopic Division of the N. N. Blokhin National Medical Research Center of Oncology, we want do demonstrate that while diagnostic radiology has achieved high quality, videoendoscopic examination remains the most objective and usually definitive method in evaluation of contentious situations of differential diagnosis even in cases of seemingly obvious clinical and radiological picture.

   Currently, a large number of reconstructive-plastic breast cancer surgeries are performed in Russia. To achieve a satisfactory aesthetic result, various reconstruction techniques are used, which include the use of silicone implants, microsurgical transplantation of flaps of various localizations. Despite the wide variety of reconstructive plastic surgeries, exposure to various negative factors can lead to both early and late postoperative complications.

   In oncologic practice, metastatic lesions in the mediastinal lymph nodes are primarily associated with lung cancer because of its high morbidity. However, lesions in mediastinal lymph nodes are quite common in the context of various other benign and malignant processes. Therefore, due to the anatomy of this zone, difficulties of visualization of specific lymph node groups, complicated differential diagnosis of benign and malignant processes, the problem of sampling biological materials from the affected area remains important and unsolved. Transesophageal endoscopic ultrasound-guided aspiration is a highly effective and safe method of diagnosis and staging of oncologic diseases associated with metastatic lesions in the mediastinal lymph nodes.

   Despite having common histological and immunophenotypic characteristics with other marginal zone lymphomas, splenic marginal zone lymphoma (SMZL) has distinctive clinical and biological features. The main indications for therapy of this disease (of primarily indolent progression) are progressive splenomegaly and/or progressive cytopenia. Current clinical management guidelines do not conclusively point to advantages of one certain therapy, and there is no universal algorithm of management of patients with newly diagnosed SMZL manifesting through progressive splenomegaly. Therefore, determination of indications for surgical treatment is an important problem. A clinical observation of a female patient with newly diagnosed advanced SMZL is presented. Due to progressive splenomegaly and hypersplenism symptoms, the patient underwent splenectomy. Personalized multidisciplinary approach allowed to relieve hypersplenism symptoms as quickly as possible, and the absence of postoperative complications with indolent disease progression significantly increased the patient’s quality of life and allowed to continue dynamic observation.

   Modern treatment programs for Hodgkin’s lymphoma include both drug antitumor therapy and radiation exposure. When conducting remote radiation therapy on the mediastinal lymph nodes, damage to the heart, thoracic aorta and lungs is possible. Such late cardiovascular complications as damage to the valvular apparatus and progressive atherosclerosis of the coronary arteries, leading to obstruction with the subsequent development of coronary heart disease, more often occurred after the use of extended radiation fields and high total focal doses. The article presents a clinical case of a 64-year-old patient with Hodgkin’s lymphoma, who successfully underwent staged cardiac surgery for severe aortic stenosis and progressive atherosclerosis with calcification of the coronary arteries due to radiation exposure.

   Background. Renal failure (RF) is the most common complication of multiple myeloma (MM), and severe RF requiring hemodialysis is diagnosed in 2–4 % of cases. RF associated with MM is potentially reversible. Severe RF requiring hemodialysis is associated with low overall survival rates, increased risk of complications and early death, low quality of life for patients.

   Aim. To analyze treatment results of newly diagnosed MM patients with severe RF requiring hemodialysis.

   Materials and methods. We analyzed data of 39 patients with newly diagnosed MM and severe RF requiring hemodialysis (CKD-EPI < 15 mL/min/1.73 m²) who underwent combination therapy at the N.N. Blokhin National Medical Research Center of Oncology between January 2000 and December 2020.

   Results. As induction therapy, 25 (64.1 %) patients received bortezomib-based regimens: 13 (33.3 %), VCD (bortezomib + cyclophosphamide + dexamethasone); 12 (30.8 %), VCP (bortezomib + cyclophosphamide + prednisolone); 14 (35.9 %) patients received chemotherapy: VAD (vincristine + doxorubicin + prednisolone), VMCP (vincristine + melphalan + cyclophosphamide + prednisolone), CD (cyclophosphamide + prednisolone). Seven (17.9 %) patients underwent high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation, 5 (71.4 %) patients from this group received bortezomib-based regimens (VCD, VCP), and 2 (28.6 %) – chemotherapy (VAD). After induction therapy, 19 (48.7 %) patients achieved objective hematologic response, and 23 (59 %) patients achieved objective renal response. The use of bortezomib-based induction therapy significantly increased the likelihood of achieving both hematologic and renal responses (p = 0.021 and p = 0.049, respectively) compared with the use of regimens without bortezomib. With a median follow-up of 19 months (95 % confidence interval 1–64 months), median progression-free survival was 15 months (95 % confidence interval: 8–26 months), median overall survival was 29 months (95 % confidence interval: 9–47 months). Multivariate analysis showed statistical significance of the effect of receiving autologous hematopoietic stem cell transplantation on progression-free survival and overall survival (p = 0.015 and p = 0.018, respectively).

   Conclusion. Using bortezomib-based regimens in newly diagnosed MM patients with severe RF requiring hemodialysis is associated with greater likelihood of achieving both hematologic and renal responses. Performing autologous hematopoietic stem cell transplantation improves progression-free survival and overall survival.

   Generalized peritonitis is an acute surgical condition characterized by high risk of complications and death. The key priority in management of patients with peritonitis is early surgical intervention and control of the source of infection. This literature review aims to highlight the modern approaches to surgical and anaesthetic management of patients with acute generalised peritonitis.