<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE root>
<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">MD-Onco</journal-id><journal-title-group><journal-title xml:lang="en">MD-Onco</journal-title><trans-title-group xml:lang="ru"><trans-title>MD-Onco</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2782-3202</issn><issn publication-format="electronic">2782-6171</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">116</article-id><article-id pub-id-type="doi">10.17650/2782-3202-2024-4-2-65-70</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>RARE AND COMPLEX CLINICAL SITUATIONS: DIAGNOSIS AND SELECTION OF TREATMENT TACTICS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>РЕДКИЕ И СЛОЖНЫЕ КЛИНИЧЕСКИЕ СИТУАЦИИ: ДИАГНОСТИКА И ВЫБОР ТАКТИКИ ЛЕЧЕНИЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Acute promyelocytic leukemia presenting with basophilia: diagnostic features and clinical observation</article-title><trans-title-group xml:lang="ru"><trans-title>Острый промиелоцитарный лейкоз, протекающий с базофилией: особенности диагностики и клиническое наблюдение</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0005-0518-3621</contrib-id><name-alternatives><name xml:lang="en"><surname>Akezheva</surname><given-names>K. A.</given-names></name><name xml:lang="ru"><surname>Акежева</surname><given-names>К. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><bold>Karina A. Akezheva</bold> </p><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><bold>Карина Амирбиевна Акежева </bold></p><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><email>akezheva@list.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9969-8482</contrib-id><name-alternatives><name xml:lang="en"><surname>Aleshina</surname><given-names>O. A.</given-names></name><name xml:lang="ru"><surname>Алешина</surname><given-names>О. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4316-4833</contrib-id><name-alternatives><name xml:lang="en"><surname>Vasileva</surname><given-names>A. N.</given-names></name><name xml:lang="ru"><surname>Васильева</surname><given-names>А. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9877-0796</contrib-id><name-alternatives><name xml:lang="en"><surname>Dvirnyk</surname><given-names>V. N.</given-names></name><name xml:lang="ru"><surname>Двирнык</surname><given-names>В. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Ershov</surname><given-names>A. A.</given-names></name><name xml:lang="ru"><surname>Ершов</surname><given-names>А. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1613-652X</contrib-id><name-alternatives><name xml:lang="en"><surname>Obukhova</surname><given-names>T. N.</given-names></name><name xml:lang="ru"><surname>Обухова</surname><given-names>Т. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1082-8659</contrib-id><name-alternatives><name xml:lang="en"><surname>Kovrigin</surname><given-names>A. M.</given-names></name><name xml:lang="ru"><surname>Ковригина</surname><given-names>А. М.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4827-8947</contrib-id><name-alternatives><name xml:lang="en"><surname>Troitskaya</surname><given-names>V. V.</given-names></name><name xml:lang="ru"><surname>Троицкая</surname><given-names>В. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>4 Novy Zykovskiy Proezd, Moscow 125167</italic></p></bio><bio xml:lang="ru"><p><italic>125167 Москва, Новый Зыковский проезд, 4</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">National Medical Research Center for Hematology</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2024-06-14" publication-format="electronic"><day>14</day><month>06</month><year>2024</year></pub-date><volume>4</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>65</fpage><lpage>70</lpage><history><date date-type="received" iso-8601-date="2024-06-14"><day>14</day><month>06</month><year>2024</year></date><date date-type="accepted" iso-8601-date="2024-06-14"><day>14</day><month>06</month><year>2024</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2024, ABV-Press</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2024, АБВ-пресс</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="en">ABV-Press</copyright-holder><copyright-holder xml:lang="ru">АБВ-пресс</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://mdonco.abvpress.ru/jour/about/editorialPolicies</ali:license_ref></license></permissions><self-uri xlink:href="https://mdonco.abvpress.ru/jour/article/view/116">https://mdonco.abvpress.ru/jour/article/view/116</self-uri><abstract xml:lang="en"><p>Acute promyelocytic leukemia (APL) is a blood tumor characterized by aggressive clinical progression with characteristic clinical and laboratory signs (typical tumor cell morphology, severe hemorrhagic syndrome, hemorrhages with hematoma formation, excessive fibrinolysis, disseminated intravascular coagulation syndrome) but modern approaches to therapy potentially make this disease fully curable. APL is considered one of the most studied subtypes of acute leukemia and is characterized by abnormal proliferation of promyelocytes in the bone marrow. The morphologic picture of APL is quite diverse, but an increased basophil count represents a rare manifestation that requires further study and understanding of its significance. Currently, there are limited scientific data on the clinical course and response to standard therapy in patients with APL presenting with basophilia.</p></abstract><trans-abstract xml:lang="ru"><p>Острый промиелоцитарный лейкоз (ОПЛ) – опухолевое заболевание крови, которое отличается агрессивным клиническим течением с характерными клинико-лабораторными признаками (типичная морфология опухолевых клеток, тяжелый геморрагический синдром, гематомный тип кровоточивости, избыточно активированный фибринолиз, синдром диссеминированного внутрисосудистого свертывания крови), но при использовании современных подходов терапии является потенциально полностью излечимым. ОПЛ считается одним из наиболее изученных подтипов острых лейкозов и характеризуется аномальной пролиферацией промиелоцитов в костном мозге. Морфологическая картина ОПЛ достаточно разнообразна, однако повышение числа базофилов представляет собой редкое проявление, требующее дополнительного изучения и понимания его значения. В настоящее время имеется ограниченное количество научных данных о клиническом течении заболевания и ответе на стандартную терапию у пациентов с ОПЛ, протекающим с базофилией.</p><p> </p></trans-abstract><kwd-group xml:lang="en"><kwd>acute promyelocytic leukemia</kwd><kwd>basophilia</kwd><kwd>systemic mastocytosis</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>острый промиелоцитарный лейкоз</kwd><kwd>базофилия</kwd><kwd>системный мастоцитоз</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Hillestad L.K. Acute promyelocytic leukemia. Acta Med Scand 1957; 159(3):189–94.</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Lo-Coco F., Hasan S.K. Understanding the molecular pathogenesis of acute promyelocytic leukemia. Best Pract Res Clin Haematol 2014;27(1):3–9. DOI: 10.1016/j.beha.2014.04.006</mixed-citation></ref><ref id="B3"><label>3.</label><mixed-citation>Mannan A., Muhsen I.N., Barragán E. et al. Genotypic and phenotypic characteristics of acute promyelocytic leukemia translocation variants. Hematol Oncol Stem Cell Ther 2020;13(4):189–201. DOI: 10.1016/j.hemonc.2020.05.007</mixed-citation></ref><ref id="B4"><label>4.</label><mixed-citation>Lo-Coco F., Di Donato L., GIMEMA, Schlenk R.F. Targeted therapy alone for acute promyelocytic leukemia. N Engl J Med 2016;374(12):1197–8. DOI: 10.1056/NEJMc1513710</mixed-citation></ref><ref id="B5"><label>5.</label><mixed-citation>Salhotra A., Mei M. Acute promyelocytic leukemia: update on risk stratification and treatment practices. Cancer Treat Res 2021;181:45–55. DOI: 10.1007/978-3-030-78311-2_3</mixed-citation></ref><ref id="B6"><label>6.</label><citation-alternatives><mixed-citation xml:lang="en">Acute promyelocytic leukemia. Clinical recommendations of the Ministry of Health Russia. 2020. Available at: https://cr.minzdrav.gov.ru/schema/132_1?ysclid=lwgbknpntg696196868</mixed-citation><mixed-citation xml:lang="ru">Острый промиелоцитарный лейкоз. Клинические рекомендации Минздрава России. 2020. Доступно по: https://cr.minzdrav.gov.ru/schema/132_1?ysclid=lwgbknpntg696196868</mixed-citation></citation-alternatives></ref><ref id="B7"><label>7.</label><mixed-citation>Rieu J.B., Canali A., Thene E. et al. Acute promyelocytic leukaemia associated with atypical basophilia. Br J Haematol 2023;201(6):1017. DOI: 10.1111/bjh.18793</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Tallman M.S., Hakimian D., Snower D. et al. Basophilic differentiation in acute promyelocytic leukemia. Leukemia 1993;7(4):521–6.</mixed-citation></ref><ref id="B9"><label>9.</label><mixed-citation>Kahn P., Plementas H. [Case of basophilic promyelocytic-I-leukemia (In German)]. Wien Z Inn Med 1971;52(12):578–583.</mixed-citation></ref><ref id="B10"><label>10.</label><mixed-citation>Matarraz S., Leoz P., Fernández C. et al. Basophil-lineage commitment in acute promyelocytic leukemia predicts for severe bleeding after starting therapy. Mod Pathol 2018;31(8):1318–31. DOI: 10.1038/s41379-018-0038-2</mixed-citation></ref></ref-list></back></article>
