Pub Date : 2023-02-14DOI: 10.24287/1726-1708-2023-22-1-156-164
M. Senchenko, D. Abramov, N. Myakova, D. Konovalov
Progressive transformation of germinal centers (PTGC) is a benign reactive lymphadenopathy, which can be an independent disease or occur in association with other lymphomas, the most common variant of which is nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). To date, it has not been definitively clarified how PTGC and NLPHL are interconnected, despite the abundance of works presented on this topic. PTGC may precede NLPHL, occur synchronously with it, or develop after a course of therapy in patients with NLPHL. Despite similar clinical and morphological features, the approach to the treatment and management of patients is different. In the case of NLPHL, one of the therapeutic options is chemotherapy, which is not used in patients with PTGC. This article presents a clinical case of partial lymph node lesion of NLPHL associated with PTGC, on the example of which the main issues of differential diagnosis of PTGC and NLHLP will be considered. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.
{"title":"Progressive transformation of germinal centers or nodular lymphocyte-predominant Hodgkin lymphoma? Issues of differential diagnosis: a clinical case","authors":"M. Senchenko, D. Abramov, N. Myakova, D. Konovalov","doi":"10.24287/1726-1708-2023-22-1-156-164","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-1-156-164","url":null,"abstract":"Progressive transformation of germinal centers (PTGC) is a benign reactive lymphadenopathy, which can be an independent disease or occur in association with other lymphomas, the most common variant of which is nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). To date, it has not been definitively clarified how PTGC and NLPHL are interconnected, despite the abundance of works presented on this topic. PTGC may precede NLPHL, occur synchronously with it, or develop after a course of therapy in patients with NLPHL. Despite similar clinical and morphological features, the approach to the treatment and management of patients is different. In the case of NLPHL, one of the therapeutic options is chemotherapy, which is not used in patients with PTGC. This article presents a clinical case of partial lymph node lesion of NLPHL associated with PTGC, on the example of which the main issues of differential diagnosis of PTGC and NLHLP will be considered. The patient's parents gave their 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":"13 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90939076","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}
Pub Date : 2023-02-14DOI: 10.24287/1726-1708-2023-22-1-46-52
D. Evstratov, A. D. Shutova, Y. Y. Dyakonova, S. Radygina, Y. Abugova, L. K. Anderzhanova, L. A. Vavilova, D. Litvinov, G. Novichkova, A. Popov, V. Fominykh, L. Khachatryan, L. Shelikhova, N. Myakova
Today, treatment results for acute lymphoblastic leukemia (ALL) look encouraging, yet 10–15% patients still end up relapsing. The success of relapse treatment is directly dependent on whether or not a tumor clone has been completely eradicated before hematopoietic stem cell transplantation (HSCT). Immunotherapy made it possible to achieve minimal residual disease (MRD) – negative remission even in refractory patients. One example of such immunotherapeutic agents is inotuzumab ozogamicin (InO), an anti-CD22 monoclonal antibody conjugated to the cytotoxic agent calicheamicin. We included 17 patients under the age of 18 with relapsed or refractory precursor B-cell ALL (pre-B ALL) who had been treated with InO at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia from 01.10.2016 to 01.09.2022. 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 efficacy of the therapy was assessed based on the patients’ morphological response, MRD negativity and overall survival. Treatment toxicity was assessed according to CTCAE 5.0 (Common Terminology Criteria for Adverse Events). Statistical analysis was performed using the XLSTAT 2016 software. The majority of the patients (75%) responded to the therapy. MRD negativity was achieved in 41.2% of the study patients. The one-year overall survival rate was 40.3% (95% confidence interval 14.8–65.7). The treatment was well tolerated but 33% of the patients treated with standard-dose InO and subsequent HSCT developed veno-occlusive disease/sinusoidal obstruction syndrome. In our study, we demonstrated the high efficacy of InO both when used as a rescue therapy in patients with relapsed/refractory pre-B ALL and as a bridging therapy in patients before HSCT.
{"title":"The use of inotuzumab ozogamicin in children with relapsed/refractory B-lineage acute lymphoblastic leukemia","authors":"D. Evstratov, A. D. Shutova, Y. Y. Dyakonova, S. Radygina, Y. Abugova, L. K. Anderzhanova, L. A. Vavilova, D. Litvinov, G. Novichkova, A. Popov, V. Fominykh, L. Khachatryan, L. Shelikhova, N. Myakova","doi":"10.24287/1726-1708-2023-22-1-46-52","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-1-46-52","url":null,"abstract":"Today, treatment results for acute lymphoblastic leukemia (ALL) look encouraging, yet 10–15% patients still end up relapsing. The success of relapse treatment is directly dependent on whether or not a tumor clone has been completely eradicated before hematopoietic stem cell transplantation (HSCT). Immunotherapy made it possible to achieve minimal residual disease (MRD) – negative remission even in refractory patients. One example of such immunotherapeutic agents is inotuzumab ozogamicin (InO), an anti-CD22 monoclonal antibody conjugated to the cytotoxic agent calicheamicin. We included 17 patients under the age of 18 with relapsed or refractory precursor B-cell ALL (pre-B ALL) who had been treated with InO at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia from 01.10.2016 to 01.09.2022. 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 efficacy of the therapy was assessed based on the patients’ morphological response, MRD negativity and overall survival. Treatment toxicity was assessed according to CTCAE 5.0 (Common Terminology Criteria for Adverse Events). Statistical analysis was performed using the XLSTAT 2016 software. The majority of the patients (75%) responded to the therapy. MRD negativity was achieved in 41.2% of the study patients. The one-year overall survival rate was 40.3% (95% confidence interval 14.8–65.7). The treatment was well tolerated but 33% of the patients treated with standard-dose InO and subsequent HSCT developed veno-occlusive disease/sinusoidal obstruction syndrome. In our study, we demonstrated the high efficacy of InO both when used as a rescue therapy in patients with relapsed/refractory pre-B ALL and as a bridging therapy in patients before HSCT.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"51 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75268748","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}
Pub Date : 2023-02-14DOI: 10.24287/1726-1708-2023-22-1-99-109
A. Rudneva, D. Abramov, A. S. Sharlay, Y. Likar, I. N. Vorozhtsov, N. Myakova
Rosai–Dorfman disease (RDD) is a rare histiocytic disorder, which occurs at any age, can affect almost any organs and tissues, does not have pathognomonic symptoms and could be confirmed only by histological examination of the affected tissue. The article describes the successful treatment of a child with RDD with lymph nodes, nasopharynx, subcutaneous tissue, spleen and bones involvement, by multistep surgical treatment and chemotherapy. A review of the literature is provided, including recommendations for the examination and treatment of patients with RDD. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.
{"title":"Rosai–Dorfman disease (sinus histiocytosis with massive lymphadenopathy): personal observations and literature review","authors":"A. Rudneva, D. Abramov, A. S. Sharlay, Y. Likar, I. N. Vorozhtsov, N. Myakova","doi":"10.24287/1726-1708-2023-22-1-99-109","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-1-99-109","url":null,"abstract":"Rosai–Dorfman disease (RDD) is a rare histiocytic disorder, which occurs at any age, can affect almost any organs and tissues, does not have pathognomonic symptoms and could be confirmed only by histological examination of the affected tissue. The article describes the successful treatment of a child with RDD with lymph nodes, nasopharynx, subcutaneous tissue, spleen and bones involvement, by multistep surgical treatment and chemotherapy. A review of the literature is provided, including recommendations for the examination and treatment of patients with RDD. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"25 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78499515","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}
Pub Date : 2023-02-14DOI: 10.24287/1726-1708-2023-22-1-84-89
S. Soliman, D. Nofal, A. Labeeb, R. E. El Zaiat, D. Fotoh
Juvenile idiopathic arthritis (JIA) is one of the most common chronic inflammatory diseases occurring in childhood, associated with decreased bone mineral density (BMD) and increased risk of osteopenia and osteoporosis, which increases the fracture risk. Aim of the work: to assess BMD and bone turnover markers (serum osteocalcin for bone formation and C terminal telopeptide of type 1 collagen for bone resorption) in JIA patients and their relation to disease activity. This study included 50 patients with JIA (female:male – 20:30). The study was approved by the Ethical Research Committee and Institutional Review Board of the Faculty of Medicine, Menoufia University, Egypt (Approval number: 19519INTPH48). Written informed consent was obtained from each patient or the parents. These patients were diagnosed with JIA according to the criteria of classification of the International League of Associations for Rheumatology. BMD was measured by Dual-energy X-ray absorptiometry (DEXA) of the lumbar spine using the Z-score. The results were correlated with JIA disease duration, disease activity, bone turnover markers and serum level of vitamin D. Clinical disease activity was evaluated by juvenile arthritis disease activity score (JADAS-27). There was a significant negative correlation between DEXA Z-score and disease activity (p-value < 0.001), bone turnover markers (p-value < 0.001), and duration of JIA (p-value < 0.05). There was a significant difference between vitamin D level and DEXA Z-score; DEXA Z-score was lower in vitamin D deficient patients. JIA patients with higher disease activity are at a higher risk of osteopenia and osteoporosis. Well-timed and efficient treatment of JIA and proper control of disease activity may help to improve the bone status and reduce the incidence of osteoporosis. Consequently, valuable targeted interventions are essential to preserve bone health during JIA.
{"title":"Assessment of bone mineral density and bone turnover markers in patients with juvenile idiopathic arthritisy","authors":"S. Soliman, D. Nofal, A. Labeeb, R. E. El Zaiat, D. Fotoh","doi":"10.24287/1726-1708-2023-22-1-84-89","DOIUrl":"https://doi.org/10.24287/1726-1708-2023-22-1-84-89","url":null,"abstract":"Juvenile idiopathic arthritis (JIA) is one of the most common chronic inflammatory diseases occurring in childhood, associated with decreased bone mineral density (BMD) and increased risk of osteopenia and osteoporosis, which increases the fracture risk. Aim of the work: to assess BMD and bone turnover markers (serum osteocalcin for bone formation and C terminal telopeptide of type 1 collagen for bone resorption) in JIA patients and their relation to disease activity. This study included 50 patients with JIA (female:male – 20:30). The study was approved by the Ethical Research Committee and Institutional Review Board of the Faculty of Medicine, Menoufia University, Egypt (Approval number: 19519INTPH48). Written informed consent was obtained from each patient or the parents. These patients were diagnosed with JIA according to the criteria of classification of the International League of Associations for Rheumatology. BMD was measured by Dual-energy X-ray absorptiometry (DEXA) of the lumbar spine using the Z-score. The results were correlated with JIA disease duration, disease activity, bone turnover markers and serum level of vitamin D. Clinical disease activity was evaluated by juvenile arthritis disease activity score (JADAS-27). There was a significant negative correlation between DEXA Z-score and disease activity (p-value < 0.001), bone turnover markers (p-value < 0.001), and duration of JIA (p-value < 0.05). There was a significant difference between vitamin D level and DEXA Z-score; DEXA Z-score was lower in vitamin D deficient patients. JIA patients with higher disease activity are at a higher risk of osteopenia and osteoporosis. Well-timed and efficient treatment of JIA and proper control of disease activity may help to improve the bone status and reduce the incidence of osteoporosis. Consequently, valuable targeted interventions are essential to preserve bone health during JIA.","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"4 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79880654","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-142-151
P. V. Kralichkin, M. Teleshova, I. Sidorov, D. Konovalov, A. E. Druy, N. Merkulov, D. Akhaladze, A. P. Troitskaya, I. E. Volkova, T. Shamanskaya, N. Zhukov, N. Myakova, D. Kachanov
{"title":"Malignant gastrointestinal neuroectodermal tumor","authors":"P. V. Kralichkin, M. Teleshova, I. Sidorov, D. Konovalov, A. E. Druy, N. Merkulov, D. Akhaladze, A. P. Troitskaya, I. E. Volkova, T. Shamanskaya, N. Zhukov, N. Myakova, D. Kachanov","doi":"10.24287/1726-1708-2022-21-4-142-151","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-142-151","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"791 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86815772","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-70-82
N. Zhukov, L. L. Rabaeva, D. Litvinov
{"title":"Efficacy and safety of low doses of olanzapine for the prevention of nausea and vomiting in children and adolescents receiving highly emetogenic chemotherapy. The interim results of a randomized trial","authors":"N. Zhukov, L. L. Rabaeva, D. Litvinov","doi":"10.24287/1726-1708-2022-21-4-70-82","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-70-82","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"11 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88655658","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-118-123
L. Papusha, A. Merishavyan, M. Zaytseva, V. A. Degtyarev, Y. Alymova, A. Druy, A. Karachunskiy
{"title":"Chiasmatic gliomas with diencephalic syndrome in infants: challenges in the diagnosis and treatment","authors":"L. Papusha, A. Merishavyan, M. Zaytseva, V. A. Degtyarev, Y. Alymova, A. Druy, A. Karachunskiy","doi":"10.24287/1726-1708-2022-21-4-118-123","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-118-123","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89796219","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-91-98
G. B. Sagoyan, A. M. Suleymanova, I. Sidorov, S. A. Sardalova, M. Rubanskaya, A. S. Temnyy, O. A. Ignatenko, A. Odzharova, D. Konovalov, O. Kosilo, A. P. Kazantsev, S. Varfolomeeva
{"title":"A clinical case of composite pheochromocytoma in a child and a literature review","authors":"G. B. Sagoyan, A. M. Suleymanova, I. Sidorov, S. A. Sardalova, M. Rubanskaya, A. S. Temnyy, O. A. Ignatenko, A. Odzharova, D. Konovalov, O. Kosilo, A. P. Kazantsev, S. Varfolomeeva","doi":"10.24287/1726-1708-2022-21-4-91-98","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-91-98","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"37 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75590633","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-163-168
M. E. Leontyeva, D. V. Bogdanova, A. Moiseeva, V. Burlakov, Z. Nesterenko, A. Y. Merkushov, N. Kan, A. Khoreva, Y. Rodina, O. Shvets, E. Deordieva, N. Kuzmenko, A. Mukhina, I. Mersiyanova, E. Raikina, A. Kozlova
{"title":"A case report of autoinflammation and PLCy2-associated antibody deficiency and immune dysregulation","authors":"M. E. Leontyeva, D. V. Bogdanova, A. Moiseeva, V. Burlakov, Z. Nesterenko, A. Y. Merkushov, N. Kan, A. Khoreva, Y. Rodina, O. Shvets, E. Deordieva, N. Kuzmenko, A. Mukhina, I. Mersiyanova, E. Raikina, A. Kozlova","doi":"10.24287/1726-1708-2022-21-4-163-168","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-163-168","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"422 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84928834","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}
Pub Date : 2022-12-26DOI: 10.24287/1726-1708-2022-21-4-152-157
A. V. Tarakanova, A. S. Sharlay, A. Panferova, I. Sidorov, D. Konovalov
{"title":"Spindle cell neoplasm harboring MAPK signaling pathway gene translocation from the spectrum of NTRK-rearranged spindle cell tumor","authors":"A. V. Tarakanova, A. S. Sharlay, A. Panferova, I. Sidorov, D. Konovalov","doi":"10.24287/1726-1708-2022-21-4-152-157","DOIUrl":"https://doi.org/10.24287/1726-1708-2022-21-4-152-157","url":null,"abstract":"","PeriodicalId":38370,"journal":{"name":"Pediatric Hematology/Oncology and Immunopathology","volume":"119 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81623544","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}
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