Pediatric Hematology/Oncology and Immunopathology最新文献

{"title":"Kaposiform lymphangiomatosis with Kasabach–Merritt phenomenon","authors":"L. Khachatryan, G. Novichkova, M. S. Vasilieva, I. Kletskaya, A. Scherbakov, A. Maschan","doi":"10.24287/1726-1708-2023-22-2-142-151","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-142-151","url":null,"abstract":"   Kaposiform lymphangiomatosis (KLA) is an aggressive lymphatic anomaly associated with bone involvement, serositis occurring at various sites, the development of Kasabach–Merritt phenomenon, and frequent infectious complications. The International Society for the Study of Vascular Anomalies classifies KLA as a subtype of generalized lymphatic anomaly. The mTOR-inhibitor rapamycin in combination with symptomatic treatment is the most common specific treatment. However, there are no standard approaches to the management of KLA. Even with modern diagnostic tools and combination therapy, the 5-year survival rate is 51 %, and the average life expectancy is 2.75 years. This article presents a classic case of KLA associated with Kasabach–Merritt phenomenon that was successfully managed with rapamycin and a liposomal form of doxorubicin as specific therapy. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"101 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88610245","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":"Pathogenesis, treatment and prevention of diseases caused by Epstein–Barr virus","authors":"A. G. Rumyantsev","doi":"10.24287/1726-1708-2023-22-2-166-174","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-166-174","url":null,"abstract":"   Studying diseases associated with viruses belonging to the family of Herpesviridae is an important challenge for medical researchers and clinicians because of the specific tropism of herpesviruses for immune cells, life-long persistence in human target cells, the ability to reactivate and the potential to cause a wide variety of clinical manifestations. Unlike other members of Herpesviridae, Epstein–Barr virus (EBV), also known as human herpes 4, displays tropism for B cells and mucosal epithelial cells, has the capacity to cause not only productive infection (infectious mononucleosis), but also establish various types of latency in cells, causes benign and malignant transformation of immune system cells (hemoblastoses) and mucosal epithelial cells (oral cavity cancer and gastric cancer). EBV causes 200 000 deaths worldwide every year, the majority of which are attributable to cancers associated with EBV persistence. Moreover, EBV is associated with a group of autoimmune disorders, such as multiple sclerosis, and secondary immunodeficiencies occurring in patients with infection of immune system cells. Mechanisms of the interaction between EBV and human cells implicated in cancer induction should be a focus of further research in fundamental virology, oncology and medicine as a whole. The interactions between EBV and target cells in mother-fetus-child system appear to be the most complicated. The inevitability of facing the virus and associated long-term consequences is determined by the time and mode of mother-to-child transmission of EBV, the presence of innate immune defense factors, genetics and molecular mechanisms of EBV latency. Recent scientific insights allow us to establish control over the evolution of EBV interactions with its host and to identify promising approaches to the prevention and treatment of previously incurable diseases associated with EBV.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"33 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81473137","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":"Guidelines for the flow cytometric minimal residual disease monitoring in B-lineage acute lymphoblastic leukemia after CD19-directed immunotherapy","authors":"E. Mikhailova, O. Illarionova, M. Maschan, G. Novichkova, A. Karachunskiy, A. Popov","doi":"10.24287/1726-1708-2023-22-2-175-184","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-175-184","url":null,"abstract":"   Multicolor flow cytometry is now routinely used in laboratory practice for the minimal residual disease (MRD) monitoring in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Wide application of CD19-directed immunotherapy leads to frequent loss of CD19 expression, that hampers significantly the flow cytometric MRD detection methodology. We developed an antibody panel and data analysis algorithm for multicolor flow cytometry, which is a reliable method for MRD detection in patients with BCP-ALL treated with CD19-directed therapy. We recommend a single-tube 11-color panel for MRD detection, which is adapted for the case of possible CD19 loss. Based on patterns of antigen expression changes and the relative expansion of normal CD19-negative BCPs, guidelines for multicolored flow cytometry data analysis and interpretation are established. The recommended approach is reliable tool for therapy response monitoring displaying the same effectiveness with the more laborious and costly molecular techniques.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"31 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76639589","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 prognostic value of HLA loss of heterozygosity after allogeneic hematopoietic stem cell transplantation in children with relapsed acute leukemia","authors":"L. Tsvetkova, A. V. Evdokimov, I. Barkhatov, O. V. Paina, O. Epifanovskaya, E. Babenko, N. Ivanova, Z. Rakhmanova, P. Kozhokar, A. Frolova, A. Osipova, S. V. Ryabenko, D. V. Kozlov, T. Gindina, E. Semenova, A. Kulagin, L. S. Zubarovskaya","doi":"10.24287/1726-1708-2023-22-2-44-53","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-44-53","url":null,"abstract":"   The loss of a patient-specific HLA haplotype on the surface of the blast cell population is one of the ways a tumor can evade the immune surveillance of donor cells. This phenomenon is observed in approximately 30 % of relapses in patients with hematologic malignancies who underwent partially mismatched allogeneic hematopoietic stem cell transplantation (HSCT). In this study, for the first time, a large cohort of pediatric patients (n = 80) with relapsed acute myeloid (AML) or acute lymphoblastic (ALL) leukemia after allogeneic HSCT was analyzed with the help of the STR method (highlypolymorphic microsatellite marker analysis) using 6 HLA haplotype markers. The study was approved by the Independent Ethics Committee and the Scientific Council of the I. P. Pavlov First Saint Petersburg State Medical University of Ministry of Healthcare of the Russian Federation. Loss of heterozygosity (LOH) was observed in 18 / 80 (22 %) relapsed patients with various types of acute leukemia: out of these, 8 / 44 (18 %) patients had B-cell ALL, 4 / 10 (40 %) patients – T-cell ALL and 6 / 25 (24 %) patients – AML. All relapses with LOH were observed in patients who had undergone haploidentical HSCT, and were found to occur later than relapses without loss of the HLA haplotype (median time to relapse: 8.8 months vs 6.2 months, p = 0.043). In the patients treated with haploidentical HSCT (n = 61), we assessed factors increasing the risk of LOH at relapse. The number of previous therapy lines in the patients with AML (n = 17) and acute or chronic graft-versus-host disease in the patients with ALL (n = 44) were associated with an increasedrisk of genetic loss of the HLA haplotype (p = 0.008 and p = 0.015 respectively). A relapse following the second allogeneic HSCT was associated with LOH in 4 / 5 (80 %) patients, p = 0.008. The prognosis of the patients with LOH was extremely poor. At a median follow-up of 6 months, the overall survival from relapse was 22 % in the LOH group and 37 % in the non-LOH group. The median overall survival was 4.5 months (95 % confidence interval 3–NA) and 10.3 months (95 % confidence interval 5.7–16.1) respectively, p = 0.063. Among the patients with LOH, the best survival rates were observed in those who had undergone a repeat allogeneic HSCT from a different donor. Thus, the analysis of LOH is an important tool for determining the prognosis and further treatment in pediatric patients with acute leukemias. We strongly recommend that this diagnostic test should be included into standard testing of patients after partially-mismatched allogeneic HSCT.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"46 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83956630","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":"Daratumumab in the treatment of pure red cell aplasia after pediatric allogeneic stem cell transplantation","authors":"S. Arakelyan, S. Blagov, S. I. Kovrygin, A. Semchenkova, M. Fadeeva, D. Evseev, T. Salimova, D. Baidildina, L. Shelikhova, M. Maschan, A. Maschan","doi":"10.24287/1726-1708-2023-22-2-104-112","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-104-112","url":null,"abstract":"Pure red cell aplasia (PRCA) is a rare complication of AB0-incompatible allogeneic hematopoietic stem cell transplantation, which manifests as a partial or complete absence of erythroid lineage in recipients with normal function of other hematopoietic lineages. There is a hypothesis, that lysis of erythroid precursors occurs because of antibody formation by population of residual B-lymphocytes and/or long-lived recipient’s plasma cells, which are capable for proliferation and active expression of the CD38 marker. That is why the invention of the IgG1 monoclonal antibody to CD38 presented as a new potentially effective targeted therapeutic option for patients with refractory PPCA. The article summarize clinical data on daratumumab for the therapy of PRCA in pediatric allogeneic hematopoietic stem cell transplantation recipients. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77712866","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 effects of correction of vitamin D deficiency in children undergoing hematopoietic stem cell transplantation","authors":"D. E. Klevakin, L. Vakhonina, D. E. Kostenko, L. Fechina","doi":"10.24287/1726-1708-2023-22-2-54-59","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-54-59","url":null,"abstract":"   Literature data suggest that vitamin D (VD) deficiency may adversely affect many systems of the body, not only skeletal system, as believed earlier, but also central nervous system, cardiovascular system, urinary system, and immune system, which is particularly important for patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT). Two consecutive studies of VD deficiency after allogeneic HSCT in pediatric patients showed that VD deficiency was associated with decreased overall survival. The correction of VD deficiency was also reported to be a challenge, and in some cases higher doses of VD were needed (200 IU/kg/day or more), but even with this more aggressive approach VD deficiency could persist. In this article, we present a literature review on this topic as well as our data on the management of VD deficiency and monitoring of serum 25-hydroxycholecalciferol (25-HVD) levels in 18 children undergoing allogeneic HSCT at our hospital. This study was approved by the Independent Ethics Committee of the Regional Children's Clinical Hospital (Yekaterinburg). Unfortunately, because of the small size of the patient group we were not able to obtain reliable scientific data. However, here we present our approach to the clinical management of VD deficiency, personalized dosing of VD, and safe therapeutic ranges of VD metabolites in blood. The majority of HSCT recipients in our study achieved therapeutic levels of 25-hydroxycholecalciferol. Treatment of VD deficiency in children undergoing allogeneic HSCT is a promising way to improve overall survival, but further studies are needed to develop optimal clinical strategies.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"152 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77480867","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 effectiveness of the respiratory muscles in children with oncohematological diseases after hematopoietic stem cell transplantation","authors":"T. V. Kudinova, Y. Skvortsova, D. E. Bostanov, О. F. Lukina, E. Tikhomirova, D. N. Balashov, L. Shelikhova, A. Karelin","doi":"10.24287/1726-1708-2023-22-2-60-64","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-60-64","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"20 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89274356","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":"Molecular genetic and cytofluorimetric prognostic factors in the development of acute myeloid leukemia relapse in children after allogeneic hematopoietic stem cell transplantation","authors":"Z. Rakhmanova, O. V. Paina, I. Barkhatov, A. M. Sadykov, S. Razumova, L. Tsvetkova, E. Babenko, T. Gindina, E. Semenova, L. S. Zubarovskaya","doi":"10.24287/1726-1708-2023-22-2-24-30","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-24-30","url":null,"abstract":"   Relapse of acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains one of the main causes of reduced long-term survival. Modern methods for predicting the risk of AML relapse after allo-HSCT take into account the data on the pre-transplant level of minimal residual disease (MRD) determined by flow cytometry and molecular biological studies of recurrent genetic abnormalities, which are currently widespread in clinical practice. Recent studies of the expression of genes characteristic of leukemic stem cells (LSCs) have shown prognostic significance for children with AML in relation to treatment response and the risk of relapse. The study of LSC persistence in order to predict the risk of recurrence after allo-HSCT in children with AML in addition to standard MRD detection methods may be of great importance. The aim of the work was to evaluate the impact of MRD status, both using classic methods and taking into account the genes characteristic of LSC, on the results of allo-HSCT in children with AML. The study was approved by the Independent Ethics Committee and the Scientific Council of the I.P. Pavlov First Saint Petersburg State Medical University of Ministry of Healthcare of the Russian Federation. To assess MRD using standard diagnostic methods, we analyzed the data of 95 children with AML in their 1st–2nd remission (cohort 1). MRD status was negative in 67 (70.6 %) patients; in 28 (29.4 %) children, MRD status was positive according to molecular genetic studies and / or immunophenotyping results. For pre-transplant evaluation of the expression of genes characteristic of LSC, we investigated bone marrow samples of 50 patients (cohort 2) using real-time polymerase chain reaction. The DNMT3B, GPR56, CD34, SOCS2, SPINK2, FAM30A, and ABL genes were studied by real-time polymerase chain reaction, followed by calculation of the pLSC6 value using the formula: DNMT3b × 0.189 + GPR56 × 0.054 + CD34 × 0.0171 + SOCS2 × 0.141 + SPINK2 × 0.109 + FAM30A × 0.0516. At the time of allo-HSCT, 37 (74 %) children with AML were in their 1st or 2nd remission of the disease, 13 (26 %) were out of the 1st–2nd remission. With a median follow-up of 5 years in the group of patients with a positive MRD status, determined by standard methods (cohort 1), overall survival (OS) was 67.9 % vs 73.1 % for patients with a negative MRD status (p = 0.83). The cumulative incidence of relapse was 50 % and 22 %, respectively; p = 0.012. When assessing the level of expression of genes characteristic of LSC (cohort 2), a pLSC6 level was above the median in 18/37 (49 %) patients. The results of linear regression showed that the pre-transplant level of expression of genes characteristic of LSC was not associated with the number of blasts/MRD (odds ratio 1.002; 95 % confidence interval 0.979–1.025). One-year OS rates did not differ significantly in children in the 1st–2nd remission of AML, depending on pLSC6 level: 84.2% in patients with","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"34 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81338776","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":"Acute graft-versus-host disease after allogeneic stem cell transplantation from a haploidentical donor with post-transplant cyclophosphamide administration","authors":"A. A. Bazaev, S. Kozlovskaya, E. Sultanova, Y. Skvortsova","doi":"10.24287/1726-1708-2023-22-2-98-103","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-98-103","url":null,"abstract":"   Allogeneic hematopoietic stem cell transplantation is the only curative treatment for many life-threatening diseases. Due to the difficulty of finding an HLA-identical donor, transplantation from a haploidentical donor has become a frequent alternative. This option is associated with a higher risk of graft-versus-host disease (GVHD). In this article, we present the current view on the pathogenesis, treatment and prevention of GVHD and report a clinical case of acute GVHD after allogeneic hematopoietic stem cell transplantation from a haploidentical donor with post-transplant cyclophosphamide prophylaxis. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"11 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74659569","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":"Acute graft-versus-host disease following allogeneic hematopoietic stem cell transplantation","authors":"Y. Skvortsova, A. Maschan","doi":"10.24287/1726-1708-2023-22-2-152-158","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-2-152-158","url":null,"abstract":"   Acute graft-versus-host disease (GVHD) remains a major complication of allogeneic hematopoietic stem cell transplantation determining the prognosis of hematopoietic stem cell recipients because of the impairment of multiple organs and systems and subsequent secondary immunodeficiency caused by combination immunosuppressive therapy. Current strategies are focused on the prevention of this life-threatening complication and on the timely start of treatment with appropriate selection of medications based on the knowledge of acute GVHD pathogenesis and grading as well as of risk factors for its development / progression in different patients. Here we provide a brief overview of acute GVHD and the existing approaches to its prevention and treatment.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"18 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79447829","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}