Pediatric Hematology/Oncology and Immunopathology最新文献

{"title":"Aplastic anemia in children: the current concept of differential diagnosis","authors":"O. Goronkova, A. Pavlova, E. Raykina","doi":"10.24287/1726-1708-2024-23-2-208-220","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-208-220","url":null,"abstract":"This article presents a brief overview of publications on pediatric aplastic anemia (AA) and closely related conditions. Here we consider the pathophysiology of AA, which includes three main mechanisms of bone marrow destruction resulting in aplasia: direct injury, immune mediated destruction and bone marrow failure resulting from inherited and clonal disorders. New aspects of inherited bone marrow failure syndromes, inborn errors of immunity and myelodysplastic syndromes are highlighted as the most common conditions included in the spectrum of differential diagnosis of AA in children. A comprehensive algorithm for the diagnosis of AA in children is presented, including standard laboratory tests and additional modern molecular and genetic techniques that contribute to a better understanding of this heterogeneous group of diseases and determine approaches to the choice of therapy. The purpose of the review is to provide pediatricians and pediatric hematologists with an updated information of this rare, heterogeneous condition based on an analysis of the latest literature data.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"60 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141813069","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intracellular signaling involved in the programmed neutrophil cell death leading to the release of extracellular DNA traps in thrombus formation","authors":"A. N. Sveshnikova, E. A. Adamanskaya, Yu.-D. D. Korobkina, M. A. Panteleev","doi":"10.24287/1726-1708-2024-23-2-222-230","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-222-230","url":null,"abstract":"The formation of extracellular DNA traps by neutrophils, or NETs (neutrophil extracellular traps) plays an essential role in many pathological processes related to hematological, oncological, and immunological diseases. This mechanism of the programmed cell death of neutrophils and other leukocytes appears to be also involved in the pathogenesis of thrombosis and thrombotic complications of a variety of disorders. In this review, we discuss the pathways of intracellular signaling leading to neutrophil activation in thrombosis and hemostasis. Even though the biochemical reactions in a cell are quite well investigated, the regulation of activity of specific proteins involved in NETosis, such as NADPH oxidase (NOX) and protein-arginine deiminase (PAD4), requires further investigation. Current approaches to the pharmacological modulation of NETosis are also specifically addressed here.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"67 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141812708","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The structure of hemostatic aggregate and the assessment of platelet functional activity using flow cytometry","authors":"A. Balandina, A. D. Kuprash, N. S. Nikitin, T. A. Kovalenko, A. Ignatova, F. Ataullakhanov, M. A. Panteleev","doi":"10.24287/1726-1708-2024-23-2-192-197","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-192-197","url":null,"abstract":"A thrombus is a heterogeneous structure consisting of platelets in different functional states. Flow cytometry is one of the most promising tools for the diagnosis of platelet state. However, its optimization and standardization are the subjects of heated debate. How to properly activate and label platelets in order to assess their functional status? In this work, we would like to briefly highlight this issue and propose the hypothesis that several levels/types of platelet activation correspond to various positions in the thrombus and various physiological meanings. One should use this entire necessary and sufficient set of activation levels in order to draw a conclusion about how the patient’s platelets “feel”.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"18 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141814574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A morphometric analysis of platelets using transmission electron microscopy","authors":"A. A. Kuznetsova, I. I. Kireev, S. Obydennyi","doi":"10.24287/1726-1708-2024-23-2-140-144","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-140-144","url":null,"abstract":"Platelets are the second most abundant human blood cells. They have an important function to form blood clots at sites of vascular injury to prevent bleeding. Abnormalities of platelet structure can lead to various dysfunctions and life-threatening situations. In some hereditary platelet disorders, morphological examination of platelets with transmission electron microscopy (TEM) may be required. TEM is technically complex, and its use is limited due to the need for expensive equipment and trained personnel. In our study, we assessed the morphometric parameters of platelets obtained from 20 healthy donors using TEM. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"19 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141816297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Red blood cell filterability measurement in the diagnosis of hereditary spherocytosis","authors":"D. S. Prudinnik, L. Koleva, E. Bovt, N. S. Kushnir, A. S. Suvorova, I. A. Dolgikh, S. S. Shakhidjanov, V. Vitvitsky, F. Ataullakhanov, E. Sinauridze, S. Plyasunova, N. S. Smetanina","doi":"10.24287/1726-1708-2024-23-2-145-151","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-145-151","url":null,"abstract":"The differential diagnosis of hereditary spherocytosis is a great challenge because of the similar clinical and laboratory signs it shares with other hereditary hemolytic anemias as well as due to the limited availability of molecular genetic testing. The development of easy-to-perform laboratory tests for the differential diagnosis of hereditary hemolytic anemias remains as relevant as ever. Here, a method of measuring red blood cell filterability for the diagnosis of hereditary spherocytosis is proposed for the first time. The aim of our study was to compare red blood cell filterability measurement with other diagnostic tests for hereditary spherocytosis as well as to assess its specificity and sensitivity. We included 30 patients (18 girls and 12 boys, with the median age of 8.6 years) with hereditary spherocytosis and 15 patients (9 girls and 6 boys, with the median age of 10 years) with other hereditary hemolytic anemias (pyruvate kinase deficiency (n = 14) and stomatocytosis (n = 1)). The diagnostic work-up for hereditary hemolytic anemia included a complete blood count test using an automated hematology analyzer, an osmotic resistance analysis before and after 24 hours of incubation at 37°С, erythrocytometry with sphericity index calculation, EMA test (eosin-5-maleimide binding assay) and red blood cell filterability measurement using artificial filters with cylindrical pores 3-5 μm in diameter. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The parents of all of the enrolled children signed a voluntary informed consent form for peripheral blood collection and diagnostic testing. In all the cases of hereditary spherocytosis diagnosed in accordance with the relevant clinical recommendations, red blood cell filterability was very low (0–0.31 units). It was higher only in 3 instances, reaching 0.47, 0.64 and 0.82 units, but in two of these cases there were no genetic data available, and the remaining patient was found to harbor the SPTA1 c.433999C>T mutation which was characterized both as spherocytosis and elliptocytosis. Red blood cell filterability in the group of the patients with other hemolytic anemias equalled 0.55 to 0.86 units (with the median of 0.77 units). The sensitivity of the RBC filterability measurement method in diagnosing hereditary spherocytosis was 93% (with 100% specificity), while the EMA test had a sensitivity of 89% and specificity of 95%. Our comparative study showed that red blood cell filterability measurement and the EMA test have similar sensitivity and specificity in diagnosing hereditary spherocytosis but the former is much cheaper and easier to perform since it does not require expensive equipment and can be carried out at any laboratory.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"26 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141814387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chronic graft-versus-host disease after allogeneic hematopoietic stem cell transplantation","authors":"Y. Skvortsova","doi":"10.24287/1726-1708-2024-23-2-158-166","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-158-166","url":null,"abstract":"Chronic graft-versus-host disease (GVHD) is a serious complication of allogeneic hematopoietic stem cell transplantation (HSCT) caused by immune dysregulation leading to multisystem involvement resulting in tissue sclerosis. This is a long-term complication that can significantly affect the quality of life of HSCT recipients due to secondary immunodeficiency associated with combined immunosuppressive therapy, impaired organ function and even disability. In addition to active prophylaxis for chronic GVHD, regular follow-up of patients is necessary for early detection of signs and symptoms of GVHD to enable timely and effective treatment. Here, we present a brief overview of novel approaches to diagnosis, classification, and staging of chronic GVHD, as well as current prophylaxis and treatment options.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"16 17","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141814586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The clinical course of the novel coronavirus disease in children after allogeneic hematopoietic stem cell transplantation","authors":"A. O. Vereshchagina, G. Solopova, T. Bykova, M. Popova, D. N. Balashov, N. V. Suvorova, E. V. Rozanceva, P. A. Levin, L. Zubarovskaya, G. Novichkova","doi":"10.24287/1726-1708-2024-23-2-107-115","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-107-115","url":null,"abstract":"The clinical course of the novel coronavirus disease (COVID-19) in patients with oncological and hematological diseases after hematopoietic stem cell transplantation (HSCT), are of special interest. To further investigate the problem, a two-center study was undertaken at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology and the R.M. Gorbacheva National Research Institute for Pediatric Oncology, Hematology and Transplantation between January 2020 and January 2023. This was a retrospective-prospective, non-randomized, non-interventional study that included children aged 0–19 years with oncological and hematological diseases and primary immunodeficiencies who had undergone allogeneic HSCT and subsequently contracted COVID-19. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. COVID-19 cases were confirmed by polymerase chain reaction testing and classified as asymptomatic, mild, moderate, severe, and critical. The study included 105 patients with a median age of 9 years; male patients were predominant (the male-to-female ratio was 1.8:1). The primary diseases were hematological malignancies (73%), benign hematological diseases (14%) and primary immunodeficiencies (13%). The most common clinical symptoms of COVID-19 were fever, gastrointestinal symptoms, and respiratory symptoms; 40% of COVID-19 cases were asymptomatic. Lymphopenia was found to be a risk factor for severe COVID-19. The patients without immune reconstitution had a longer persistence of the COVID-19 virus than those with immune reconstitution (17 days versus 13 days), however, no significant differences were obtained (p = 0.7). There were also no significant differences in the severity and outcomes of COVID-19 between the patients with immune reconstitution and those without reconstitution. There was no effect of therapy on the duration of COVID-19, and there was no association between the type of treatment and the duration of the disease. The overall survival rate in the allo-HSCT recipients who had been diagnosed with COVID-19 was 88%, which was lower than in the non-recipients (88% vs 94%; p = 0,077).","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"90 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141818723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complications of high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation in children with solid malignant neoplasms: a single-center experience","authors":"T. Z. Aliev, K. Kirgizov, E. Machneva, I. Kostareva, K. Sergeenko, D. S. Smirnova, N. Burlaka, Yu. V. Lozovan, I. Y. Trushkova, A. Elfimova, K. V. Mitrakov, T. I. Potemkina, M. D. Malova, R. R. Fatkhullin, N. Stepanyan, D. A. Kapkova, G. Sagoyan, A. M. Suleymanova, N. Matinyan, G. Muftakhova, A. P. Kazantsev, O. M. Romantsova, M. Rubanskaya, T. Ushakova, A. Rodina, V. V. Zhogov, V. S. Vanesyan, Y. Skvortsova, I. Kazantsev, A. S. Slinin, T. Gorbunova, T. T. Valiev, V. G. Polyakov, S. Varfolomeeva","doi":"10.24287/1726-1708-2024-23-2-116-127","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-2-116-127","url":null,"abstract":"High-dose chemotherapy (HDCT) followed by autologous hematopoietic stem cell transplantation (auto-HSCT) is a therapeutic option that allows potentiating the antitumor effect in patients with malignant neoplasms (MNs) belonging to the high-risk group. However, despite the effectiveness of this method, the risks of developing infectious and toxic complications in the early and late post-transplantation period are higher than the risks associated with treatment according to standard protocols and can significantly worsen the results of transplantation. We carried out a retrospective analysis of the results of auto-HSCT in a cohort of 156 patients with high-risk solid MNs treated at the L.A. Durnov Research Institute of Pediatric Oncology and Hematology, the N.N. Blokhin National Medical Research Center of Oncology of Ministry of Healthcare of the Russian Federation in 2020–2023. The study was approved by the Independent Ethics Committee and the Scientific Council of the N.N. Blokhin National Medical Research Center of Oncology. The study included 78 (50%) boys and 78 (50%) girls, the median age of the patients was 8 years 7 months (9 months – 17 years 8 months). Auto-HSCT was performed in 90 (57.7%) patients with neuroblastoma, 25 (16.0%) – with Ewing's sarcoma, 16 (10.3%) – with germ cell tumors, 13 (8.4%) – with nephroblastoma, 7 (4.5%) – with retinoblastoma, 3 (1.9%) – with medulloblastoma, 1 (0.6%) patient with pleuropulmonary blastoma and 1 (0.6%) patient with sialoblastoma. We used the following conditioning regimens: treosulfan + melphalan (n = 116), carboplatin + thiotepa + etoposide (n = 17), melphalan (n = 13), carboplatin + thiotepa + etoposide + cyclophosphamide (n = 10). Depending on the clinical indications and the treatment protocol used, 136 (87.2%) patients underwent one course of HDCT, and 20 (12.8%) patients underwent tandem HDCT. In most patients, the median recovery time for granulocytes and platelets was 11 (8–19) days and 14 (12–21) days, respectively. The most common infectious complications in patients after auto-HSCT were mucositis (89.1%), neutropenic enterocolitis (76.9%), febrile neutropenia (71.2%), less often: catheter-associated bloodstream infection (9%), pneumonia (14.1%), acute respiratory distress syndrome (0.6%). As regards toxic complications, all patients had emetic syndrome, 98 (62.8%) had dermatological toxicity, 9 (5.8%) had hemorrhagic cystitis, 116 (74.3%) had hepatic toxicity, 14 (9%) had neurotoxicity, 102 (65.4%) had moderate nutritional insufficiency. Episodes of hemorrhagic syndrome due to thrombocytopenia were observed in 44.2% of patients. After auto-HSCT, most patients develop chemotherapy-induced (including infectious) complications, which can not only significantly disrupt the patients’ well-being and quality of life, but also, depending on the severity, pose a threat to their life. The correct choice of conditioning regimen, effective collection of hematopoietic stem cells, complex accompanying","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"98 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141818593","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Conditions for the implementation of the phenomenon of programmed death of neutrophils with the appearance of DNA extracellular traps during thrombus formation","authors":"A. N. Sveshnikova, E. A. Adamanskaya, M. A. Panteleev","doi":"10.24287/1726-1708-2024-23-1-211-218","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-1-211-218","url":null,"abstract":"   The formation of DNA extracellular traps of neutrophils (NET-osis) is a mechanism of programmed cell death of leukocytes, which initially has antibacterial and antifungal functions. The ability of neutrophils to become activated upon contact with activated platelets and, in turn, to activate the contact coagulation pathway via DNA traps plays a central role in venous thrombosis and disseminated intravascular coagulation in COVID-19. At the same time, the intracellular signaling that controls NET-osis is extremely poorly understood even for the simplest cases, when this process is caused by lipopolysaccharides of the bacterial cell wall. In this review, we consider the case of NET-osis in thrombosis, for which there are even more questions. We focused on the conditions for NET-osis observation and features in different scenarios.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":" 29","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140683892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunophenotyping in the diagnosis of acute leukemias of ambiguous lineage. The results of centralized diagnosis and practical guidelines","authors":"I. A. Demina, E. Mikhailova, A. Semchenkova, T. Verzhbitskaya, Zh. V. Permikin, S. Kashpor, E. Zerkalenkova, G. Tsaur, Y. Olshanskaya, L. Fechina, A. Karachunskiy, G. Novichkova, A. Popov","doi":"10.24287/1726-1708-2024-23-1-219-230","DOIUrl":"https://doi.org/10.24287/1726-1708-2024-23-1-219-230","url":null,"abstract":"   Acute leukemias of ambiguous lineage (ALAL) are rare acute leukemias (AL) that exhibit specific features of more than one hematopoietic lineage or show no distinct evidence of lineage differentiation. Immunophenotyping plays a key role in the diagnosis and classification of ALAL. Despite the availability of diagnostic criteria for ALAL proposed by different expert groups, the accurate diagnosis of ALAL representing a rare and heterogeneous group of diseases remains a challenge. In this paper, we present a brief analysis of 97 pediatric ALAL cases. Such a large cohort of cases with ALAL (ALALs comprising less than 1 % of all pediatric AL) was obtained as a result of the centralized diagnosis of AL. With regard to the obtained results, we have developed the guidelines for the interpretation of the results of immunophenotyping in the diagnosis of ALAL and for the integration of findings from flow cytometry, cytomorphology and genetic testing for the accurate diagnosis and classification of this group of AL.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":" January","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140682931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}