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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Pediatric Hematology/Oncology and Immunopathology</journal-id><journal-title-group><journal-title xml:lang="en">Pediatric Hematology/Oncology and Immunopathology</journal-title><trans-title-group xml:lang="ru"><trans-title>Вопросы гематологии/онкологии и иммунопатологии в педиатрии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1726-1708</issn><issn publication-format="electronic">2414-9314</issn><publisher><publisher-name xml:lang="en">Fund Doctors, Innovations, Science for Children</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">387</article-id><article-id pub-id-type="doi">10.24287/1726-1708-2020-19-3-84-89</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>CLINICAL OBSERVATIONS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Features of relapses and refractory forms of T-lymphoblastic lymphoma in children</article-title><trans-title-group xml:lang="ru"><trans-title>Особенности течения рецидивов и рефрактерных форм Т-лимфобластной лимфомы у детей</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6314-2132</contrib-id><name-alternatives><name xml:lang="en"><surname>Abashidze</surname><given-names>Z. A.</given-names></name><name xml:lang="ru"><surname>Абашидзе</surname><given-names>З. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Hematologist Department of pediatric hematology, oncology, </p><p>117997, Moscow, Samory Mashela st., 1</p></bio><bio xml:lang="ru"><p>врач-гематолог боксированного отделения гематологии/онкологии,</p><p>117997, Москва, ул. Саморы Машела, 1 </p></bio><email>zalina.abashidze@fccho-moscow.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8725-7532</contrib-id><name-alternatives><name xml:lang="en"><surname>Dyakonova</surname><given-names>U. U.</given-names></name><name xml:lang="ru"><surname>Дьяконова</surname><given-names>Ю. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4779-1896</contrib-id><name-alternatives><name xml:lang="en"><surname>Myakova</surname><given-names>N. V.</given-names></name><name xml:lang="ru"><surname>Мякова</surname><given-names>Н. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1</p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology, Ministry of Healthcare of Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии&#13;
и иммунологии им. Дмитрия Рогачева» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2020-09-08" publication-format="electronic"><day>08</day><month>09</month><year>2020</year></pub-date><volume>19</volume><issue>3</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>84</fpage><lpage>89</lpage><history><date date-type="received" iso-8601-date="2020-10-05"><day>05</day><month>10</month><year>2020</year></date><date date-type="accepted" iso-8601-date="2020-10-05"><day>05</day><month>10</month><year>2020</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2025, «D. Rogachev NMRCPHOI»</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2025, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="en">«D. Rogachev NMRCPHOI»</copyright-holder><copyright-holder xml:lang="ru">ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://hemoncim.com/jour/article/view/387">https://hemoncim.com/jour/article/view/387</self-uri><abstract xml:lang="en"><p>Lymphoblastic lymphoma (LBL) is a rapidly progressive, malignant disease from T and B progenitor cells. Lymphomas from T cell precursors (T-LBL) account for up to 80% of all LBLs. Despite the rather rare occurrence of T-LBL, the relapsing and refractory course of this disease is an actual problem. Programs for the treatment of relapses and refractory forms of T-LBL are currently being actively developing. The role and place of targeted drugs in the multimodal T-LBL relapse strategy is determined. Further fundamental research is aimed at overcoming drug resistance, studying the molecular genetic mechanisms, tumor cell signaling pathways, which will improve treatment outcomes and survival. Based on clinical case the authors will be considered clinical features of relapses and refractory form of T-LBL and possible methods of treatment. Parents gave their consent to use information about the child, including fotos, in the article.</p></abstract><trans-abstract xml:lang="ru"><p>Лимфобластная лимфома (ЛБЛ) является быстро прогрессирующим злокачественным заболеванием из предшественников Т- и В-клеток. На долю лимфом из Т-клеток-предшественников (Т-ЛБЛ) приходится до 80% всех ЛБЛ. Несмотря на достаточно редкую встречаемость Т-ЛБЛ, рецидивирующее и рефрактерное течение данного заболевания представляет собой актуальную проблему. Программы терапии рецидивов и рефрактерных форм Т-ЛБЛ в настоящее время продолжают активно разрабатываться. Определяются роль и место таргетных препаратов в мультимодальной стратегии лечения рецидивов Т-ЛБЛ. Дальнейшие фундаментальные исследования направлены на преодоление лекарственной резистентности, изучение молекулярногенетических механизмов, сигнальных путей опухолевой клетки, что позволит улучшить результаты лечения и выживаемость. В статье на примере клинического случая рассмотрены особенности течения рецидивов и рефрактерных форм Т-ЛБЛ у детей, а также возможные методы терапии. Родители дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.</p></trans-abstract><kwd-group xml:lang="en"><kwd>relapse</kwd><kwd>T-lymphoblastic lymphoma</kwd><kwd>signaling pathways</kwd><kwd>survival</kwd><kwd>prognosis</kwd><kwd>children</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>рецидив</kwd><kwd>Т-лимфобластная лимфома</kwd><kwd>сигнальные пути</kwd><kwd>выживаемость</kwd><kwd>прогноз</kwd><kwd>дети</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">1. Burkhardt B., Reiter A., Landmann E., Lang P., Lassay L., Dickerhoff R., et al. Poor outcome for children and adolescents with progressive disease or relapse of lymphoblastic lymphoma: A report from the Berlin‐Frankfurt‐Muenster group. J Clin Oncol 2009; 27 (20): 3363–9. DOI: 10.1200/JCO.2008.19.3367</mixed-citation><mixed-citation xml:lang="ru">Burkhardt B., Reiter A., Landmann E., Lang P., Lassay L., Dickerhoff R., et al. Poor outcome for children and adolescents with progressive disease or relapse of lymphoblastic lymphoma: A report from the Berlin‐Frankfurt‐Muenster group. J Clin Oncol 2009; 27 (20): 3363–9. DOI: 10.1200/JCO.2008.19.3367</mixed-citation></citation-alternatives></ref><ref id="B2"><label>2.</label><citation-alternatives><mixed-citation xml:lang="en">2. Румянцев А.Г., Масчан А.А., Жуковская Е.В. Федеральные клинические рекомендации. Детская гематология. М.: ГЭОТАР-Медиа; 2015.</mixed-citation><mixed-citation xml:lang="ru">Румянцев А.Г., Масчан А.А., Жуковская Е.В. Федеральные клинические рекомендации. Детская гематология. М.: ГЭОТАР-Медиа; 2015.</mixed-citation></citation-alternatives></ref><ref id="B3"><label>3.</label><citation-alternatives><mixed-citation xml:lang="en">3. Burkhardt B., Mueller S., Khanam T., Perkins S.L. Current status and future directions of T-lymphoblastic lymphoma in children and adolescents. Br J Haematol 2016; 173 (4): 545–59. DOI: 10.1111/bjh.14017</mixed-citation><mixed-citation xml:lang="ru">Burkhardt B., Mueller S., Khanam T., Perkins S.L. Current status and future directions of T-lymphoblastic lymphoma in children and adolescents. Br J Haematol 2016; 173 (4): 545–59. DOI: 10.1111/bjh.14017</mixed-citation></citation-alternatives></ref><ref id="B4"><label>4.</label><citation-alternatives><mixed-citation xml:lang="en">4. El-Mallawany N.K., Frazer J.K., Van Vlierberghe P., Ferrando A. A., Perkins S., Lim M., et al. Pediatric T-and NK-cell lymphomas: New biologic insights and treatment strategies. Blood Cancer J 2012; 2(4): е65. DOI: 10.1038/bcj.2012.8</mixed-citation><mixed-citation xml:lang="ru">El-Mallawany N.K., Frazer J.K., Van Vlierberghe P., Ferrando A. A., Perkins S., Lim M., et al. Pediatric T-and NK-cell lymphomas: New biologic insights and treatment strategies. Blood Cancer J 2012; 2(4): е65. DOI: 10.1038/bcj.2012.8</mixed-citation></citation-alternatives></ref><ref id="B5"><label>5.</label><citation-alternatives><mixed-citation xml:lang="en">5. T-lymphoblastic lymphoma. World Health Organization (WHO), 2017.</mixed-citation><mixed-citation xml:lang="ru">T-lymphoblastic lymphoma. World Health Organization (WHO), 2017.</mixed-citation></citation-alternatives></ref><ref id="B6"><label>6.</label><citation-alternatives><mixed-citation xml:lang="en">6. Kobayashi R., Takimoto T., Nakazawa A., Fujita N., Akazai A., Yamato K., et al. Inferior outcomes of stage III T lymphoblastic lymphoma relative to stage IV lymphoma and T‐acute lymphoblastic leukemia: long‐term comparison of outcomes in the JACLS NHL T‐98 and ALL T‐97 protocols. Int J Hematol 2014; 99 (6): 743–9. DOI: 10.1007/s12185-014-1585-z</mixed-citation><mixed-citation xml:lang="ru">Kobayashi R., Takimoto T., Nakazawa A., Fujita N., Akazai A., Yamato K., et al. Inferior outcomes of stage III T lymphoblastic lymphoma relative to stage IV lymphoma and T‐acute lymphoblastic leukemia: long‐term comparison of outcomes in the JACLS NHL T‐98 and ALL T‐97 protocols. Int J Hematol 2014; 99 (6): 743–9. DOI: 10.1007/s12185-014-1585-z</mixed-citation></citation-alternatives></ref><ref id="B7"><label>7.</label><citation-alternatives><mixed-citation xml:lang="en">7. Kobrinsky N.L., Sposto R., Shah N.R., Anderson JR, De Laat C, Morse M., et al. Outcomes of treatment of children and adolescents with recurrent non-Hodgkin's lymphoma and Hodgkin's disease with dexamethasone, etoposide, cisplatin, cytarabine, and l-asparaginase, maintenance chemotherapy, and transplantation: Children's Cancer Group Study CCG-5912. J Clin Oncol 2001; 19(9): 2390–6.</mixed-citation><mixed-citation xml:lang="ru">Kobrinsky N.L., Sposto R., Shah N.R., Anderson JR, De Laat C, Morse M., et al. Outcomes of treatment of children and adolescents with recurrent non-Hodgkin's lymphoma and Hodgkin's disease with dexamethasone, etoposide, cisplatin, cytarabine, and l-asparaginase, maintenance chemotherapy, and transplantation: Children's Cancer Group Study CCG-5912. J Clin Oncol 2001; 19(9): 2390–6.</mixed-citation></citation-alternatives></ref><ref id="B8"><label>8.</label><citation-alternatives><mixed-citation xml:lang="en">8. Michaux K., Bergeron C., Gandemer V., Mechinaud F., Uyttebroeck A., Bertrand Y.; SFCE and the EORTC children leukemia group. Relapsed or Refractory Lymphoblastic Lymphoma in Children: Results and Analysis of 23 Patients in the EORTC 58951 and the LMT96 Protocols. Pediatr Blood Cancer 2016; 63(7): 1214–21. DOI: 10.1002/pbc.25990</mixed-citation><mixed-citation xml:lang="ru">Michaux K., Bergeron C., Gandemer V., Mechinaud F., Uyttebroeck A., Bertrand Y.; SFCE and the EORTC children leukemia group. Relapsed or Refractory Lymphoblastic Lymphoma in Children: Results and Analysis of 23 Patients in the EORTC 58951 and the LMT96 Protocols. Pediatr Blood Cancer 2016; 63(7): 1214–21. DOI: 10.1002/pbc.25990</mixed-citation></citation-alternatives></ref><ref id="B9"><label>9.</label><citation-alternatives><mixed-citation xml:lang="en">9. Callens C., Baleydier F., Lengline E., Ben Abdelali R., Petit A., Villarese P., et al. Clinical Impact of NOTCH1 and/or FBXW7 Mutations, FLASH Deletion, and TCR Status in Pediatric T-Cell Lymphoblastic Lymphoma. J Clin Oncol 2012; 30(16): 1966– 73. DOI: 10.1200/JCO.2011.39.7661</mixed-citation><mixed-citation xml:lang="ru">Callens C., Baleydier F., Lengline E., Ben Abdelali R., Petit A., Villarese P., et al. Clinical Impact of NOTCH1 and/or FBXW7 Mutations, FLASH Deletion, and TCR Status in Pediatric T-Cell Lymphoblastic Lymphoma. J Clin Oncol 2012; 30(16): 1966– 73. DOI: 10.1200/JCO.2011.39.7661</mixed-citation></citation-alternatives></ref><ref id="B10"><label>10.</label><citation-alternatives><mixed-citation xml:lang="en">10. Bonn B.R., Rohde M., Zimmermann M., Krieger D., Oschlies I., Niggli F., et al. Incidence and prognostic relevance of genetic variations in T‐cell lymphoblastic lymphoma in childhood and adolescence. Blood 2013; 121(16): 3153–60.</mixed-citation><mixed-citation xml:lang="ru">Bonn B.R., Rohde M., Zimmermann M., Krieger D., Oschlies I., Niggli F., et al. Incidence and prognostic relevance of genetic variations in T‐cell lymphoblastic lymphoma in childhood and adolescence. Blood 2013; 121(16): 3153–60.</mixed-citation></citation-alternatives></ref><ref id="B11"><label>11.</label><citation-alternatives><mixed-citation xml:lang="en">11. Balbach S.T., Makarova O., Bonn B.R., Zimmermann M., Rohde M., Oschlies I. et al. Proposal of a genetic classifier for risk group stratification in pediatric T‐cell lymphoblastic lymphoma reveals differences from adult T‐cell lymphoblastic leukemia. Leukemia 2016; 30 (4): 970-3. DOI: 10.1038/leu.2015.203.</mixed-citation><mixed-citation xml:lang="ru">Balbach S.T., Makarova O., Bonn B.R., Zimmermann M., Rohde M., Oschlies I. et al. Proposal of a genetic classifier for risk group stratification in pediatric T‐cell lymphoblastic lymphoma reveals differences from adult T‐cell lymphoblastic leukemia. Leukemia 2016; 30 (4): 970-3. DOI: 10.1038/leu.2015.203.</mixed-citation></citation-alternatives></ref><ref id="B12"><label>12.</label><citation-alternatives><mixed-citation xml:lang="en">12. Kobrinsky N.L., Sposto R., Shah N.R., Anderson J.R., DeLaat C., Morse M. et al. Outcomes of treatment of children and adolescents with recurrent non-Hodgkin's lymphoma and Hodgkin's disease with dexamethasone, etoposide, cisplatin, cytarabine, and l-asparaginase, maintenance chemotherapy, and transplantation: Children's Cancer Group Study CCG-5912. J Clin Oncol 2001; 19(9):2390–6.</mixed-citation><mixed-citation xml:lang="ru">Kobrinsky N.L., Sposto R., Shah N.R., Anderson J.R., DeLaat C., Morse M. et al. Outcomes of treatment of children and adolescents with recurrent non-Hodgkin's lymphoma and Hodgkin's disease with dexamethasone, etoposide, cisplatin, cytarabine, and l-asparaginase, maintenance chemotherapy, and transplantation: Children's Cancer Group Study CCG-5912. J Clin Oncol 2001; 19(9):2390–6.</mixed-citation></citation-alternatives></ref><ref id="B13"><label>13.</label><citation-alternatives><mixed-citation xml:lang="en">13. Harris R.E., Termuhlen A.M., Smith L.M. Lynch J., Henry M.M., Perkins S.L., et al. Autologous peripheral blood stem cell transplantation in children with refractory or relapsed lymphoma: results of Children,s Oncology Group Study A5962. Biol Blood Marrow Transplant 2011; 17(2): 249– 58. DOI: 10.1016/j.bbmt.2010.07.002</mixed-citation><mixed-citation xml:lang="ru">Harris R.E., Termuhlen A.M., Smith L.M. Lynch J., Henry M.M., Perkins S.L., et al. Autologous peripheral blood stem cell transplantation in children with refractory or relapsed lymphoma: results of Children,s Oncology Group Study A5962. Biol Blood Marrow Transplant 2011; 17(2): 249– 58. DOI: 10.1016/j.bbmt.2010.07.002</mixed-citation></citation-alternatives></ref><ref id="B14"><label>14.</label><citation-alternatives><mixed-citation xml:lang="en">14. Gross T.G., Hale G.A., He W., Camitta B.M., Sanders J.E., Cairo M.S. et al. Hematopoietic stem cell transplantation for refractory or recurrent non-Hodgkin lymphoma in children and adolescents. Biol Blood Marrow Transplant 2010; 16(2): 223–30. DOI: 10.1016/j.bbmt.2009.09.021.</mixed-citation><mixed-citation xml:lang="ru">Gross T.G., Hale G.A., He W., Camitta B.M., Sanders J.E., Cairo M.S. et al. Hematopoietic stem cell transplantation for refractory or recurrent non-Hodgkin lymphoma in children and adolescents. Biol Blood Marrow Transplant 2010; 16(2): 223–30. DOI: 10.1016/j.bbmt.2009.09.021.</mixed-citation></citation-alternatives></ref><ref id="B15"><label>15.</label><citation-alternatives><mixed-citation xml:lang="en">15. Dunsmore K.P., Devidas M., Linda S.B., Borowitz M.J., Winick N., Hunger S.P., et al. Pilot study of nelarabine in combination with intensive chemotherapy in highrisk T-cell acute lymphoblastic leukemia: a report from the Children’s Oncology Group. J Clin Oncol 2012; 30(22): 2753–9. DOI: 10.1200/JCO.2011.40.8724</mixed-citation><mixed-citation xml:lang="ru">Dunsmore K.P., Devidas M., Linda S.B., Borowitz M.J., Winick N., Hunger S.P., et al. Pilot study of nelarabine in combination with intensive chemotherapy in highrisk T-cell acute lymphoblastic leukemia: a report from the Children’s Oncology Group. J Clin Oncol 2012; 30(22): 2753–9. DOI: 10.1200/JCO.2011.40.8724</mixed-citation></citation-alternatives></ref><ref id="B16"><label>16.</label><citation-alternatives><mixed-citation xml:lang="en">16. Валиев Т.Т. Клиническая характеристика и результаты терапии рецидивов/ рефрактерных форм неходжкинских лимфом у детей: обзор литературы и анализ собственных данных. Онкогематология 2018; 13(2): 21–31. DOI: 10.17650/1818-8346-2018-13-2-21-31</mixed-citation><mixed-citation xml:lang="ru">Валиев Т.Т. Клиническая характеристика и результаты терапии рецидивов/ рефрактерных форм неходжкинских лимфом у детей: обзор литературы и анализ собственных данных. Онкогематология 2018; 13(2): 21–31. DOI: 10.17650/1818-8346-2018-13-2-21-31</mixed-citation></citation-alternatives></ref><ref id="B17"><label>17.</label><citation-alternatives><mixed-citation xml:lang="en">17. Cooper T.M., Razzouk B.I., Gerbing R., Alonzo T.A., Adlard K., Raetz E., et al. Phase I/II trial of clofarabine and cytarabine in children with relapsed/refractory acute lymphoblastic leukemia (AAML0523): a report from the Children’s Oncology Group. Pediatr Blood Cancer 2013; 60(7): 1141–7. DOI: 10.1002/pbc.24398</mixed-citation><mixed-citation xml:lang="ru">Cooper T.M., Razzouk B.I., Gerbing R., Alonzo T.A., Adlard K., Raetz E., et al. Phase I/II trial of clofarabine and cytarabine in children with relapsed/refractory acute lymphoblastic leukemia (AAML0523): a report from the Children’s Oncology Group. Pediatr Blood Cancer 2013; 60(7): 1141–7. DOI: 10.1002/pbc.24398</mixed-citation></citation-alternatives></ref><ref id="B18"><label>18.</label><citation-alternatives><mixed-citation xml:lang="en">18. Dick L.R., Fleming P.E. Building on bortezomib: second-generation proteasome inhibitors as anticancer therapy. Drug Discov Today 2010; 15 (5–6): 243–9. DOI: 10.1016/j.drudis. 2010.01.008</mixed-citation><mixed-citation xml:lang="ru">Dick L.R., Fleming P.E. Building on bortezomib: second-generation proteasome inhibitors as anticancer therapy. Drug Discov Today 2010; 15 (5–6): 243–9. DOI: 10.1016/j.drudis. 2010.01.008</mixed-citation></citation-alternatives></ref><ref id="B19"><label>19.</label><citation-alternatives><mixed-citation xml:lang="en">19. Schmidt E., Burkhardt B. Lymphoblastic lymphoma in childhood and adolescence. Pediatr Hematol Oncol 2013; 30(6): 484– 508. DOI: 10.3109/08880018.2013.789574</mixed-citation><mixed-citation xml:lang="ru">Schmidt E., Burkhardt B. Lymphoblastic lymphoma in childhood and adolescence. Pediatr Hematol Oncol 2013; 30(6): 484– 508. DOI: 10.3109/08880018.2013.789574</mixed-citation></citation-alternatives></ref></ref-list></back></article>
