<?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="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Kazan medical journal</journal-id><journal-title-group><journal-title xml:lang="en">Kazan medical journal</journal-title><trans-title-group xml:lang="ru"><trans-title>Казанский медицинский журнал</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0368-4814</issn><issn publication-format="electronic">2587-9359</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">1726</article-id><article-id pub-id-type="doi">10.17816/KMJ1726</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>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Pathogenetic treatment of the patient with splenic marginal zone lymphoma complicated by Evans syndrome</article-title><trans-title-group xml:lang="ru"><trans-title>Патогенетическое лечение пациентки с неходжкинской лимфомой маргинальной зоны селезёнки, осложнённой синдромом Эванса</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Romanenko</surname><given-names>N A</given-names></name><name xml:lang="ru"><surname>Романенко</surname><given-names>Николай Александрович</given-names></name></name-alternatives><email>rom-nik@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Bessmeltsev</surname><given-names>S S</given-names></name><name xml:lang="ru"><surname>Бессмельцев</surname><given-names>Станислав Семёнович</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Abdulkadyrov</surname><given-names>K M</given-names></name><name xml:lang="ru"><surname>Абдулкадыров</surname><given-names>Кудрат Мугутдинович</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Russian Scientific and Research Institute of Hematology and Transfusiology, Saint-Petersburg, Russia</institution></aff><aff><institution xml:lang="ru">Российский научно-исследовательский институт гематологии и трансфузиологии, г. Санкт-Петербург</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2012-10-15" publication-format="electronic"><day>15</day><month>10</month><year>2012</year></pub-date><volume>93</volume><issue>5</issue><issue-title xml:lang="en">VOL 93, NO5 (2012)</issue-title><issue-title xml:lang="ru">ТОМ 93, №5 (2012)</issue-title><fpage>843</fpage><lpage>846</lpage><history><date date-type="received" iso-8601-date="2016-03-28"><day>28</day><month>03</month><year>2016</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2012, Romanenko N.A., Bessmeltsev S.S., Abdulkadyrov K.M.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2012, Романенко Н.А., Бессмельцев С.С., Абдулкадыров К.М.</copyright-statement><copyright-year>2012</copyright-year><copyright-holder xml:lang="en">Romanenko N.A., Bessmeltsev S.S., Abdulkadyrov K.M.</copyright-holder><copyright-holder xml:lang="ru">Романенко Н.А., Бессмельцев С.С., Абдулкадыров К.М.</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/">http://creativecommons.org/licenses/by-nc-sa/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://kazanmedjournal.ru/kazanmedj/article/view/1726">https://kazanmedjournal.ru/kazanmedj/article/view/1726</self-uri><abstract xml:lang="en"><p>A case report of a patients with splenic marginal zone lymphoma, in whom clinical remission was initially achieved by 5 continuous courses of combined treatment with fludarabine 25 mg/m2 and cyclophosphamide 350 mg/m2 at days 1-3. Nevertheless, 6 months after patient developed a clinical picture of Evans syndrome, characterized by severe hemolytic anemia, thrombocytopenia and neutropenia. Corticosteroid treatment accompanied by blood transfusion was initiated, the effect was marginal and short-lasting. Switch to rituximab monotherapy allowed to archieve complete clinical and hematological remission with full blood count parameters reversal.</p></abstract><trans-abstract xml:lang="ru"><p>Представлено клиническое наблюдение больной лимфомой маргинальной зоны селезёнки, у которой после 5 курсов комплексной терапии (флударабин 25 мг/м2 и циклофосфамид 350 мг/м2 в 1-3-й дни) получена клинико-гематологическая ремиссия. Однако через 6 мес у пациентки развилась клиническая картина синдрома Эванса с тяжёлой гемолитической анемией, тромбоцитопенией и нейтропенией. Проводились курсы гормонального лечения с трансфузионной терапией. Однако эффект лечения был крайне низким и кратковременным. Смена лечения на монотерапию ритуксимабом позволила получить полную клинико-гематологическую ремиссию с восстановлением показателей крови.</p></trans-abstract><kwd-group xml:lang="en"><kwd>splenic marginal zone lymphoma</kwd><kwd>anemia</kwd><kwd>thrombocytopenia</kwd><kwd>red blood cells transfusion</kwd><kwd>erythropoietin alpha</kwd><kwd>Evans syndrome</kwd><kwd>rituximab</kwd><kwd>CD20 antibodies</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>лимфома маргинальной зоны селезёнки</kwd><kwd>анемия</kwd><kwd>тромбоцитопения</kwd><kwd>трансфузии эритроцитов</kwd><kwd>эпоэтин альфа</kwd><kwd>синдром Эванса</kwd><kwd>ритуксимаб</kwd><kwd>антитела CD20</kwd></kwd-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Поддубная И.В. Неходжкинские лимфомы. Клиническая онкогематология. Руководство для врачей / Под ред. М.А. Волковой. - М.: Медицина, 2007. - С. 724-770.</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Debiasi M., Hehnemann M., Garicochea B. Rituximab monotherapy for splenic marginal zone lymphoma with villous lymphocytes: report on long-term disease control for two patients with recurrence after splenectomy // Sao Paulo Med. J. - 2010. - Vol. 128, suppl. 6. - P. 375-377.</mixed-citation></ref><ref id="B3"><label>3.</label><mixed-citation>García-Muñoz R., Rodriguez-Otero P., Pegenaute C. et al. Splenic marginal zone lymphoma with Evans’ syndrome, autoimmunity, and peripheral gamma/delta T cells // Ann. Hematol. - 2009. - Vol. 88, N 2. - P. 177-178.</mixed-citation></ref><ref id="B4"><label>4.</label><mixed-citation>Kojima M., Sato E., Oshimi K. et al. Characteristics of CD5-positive splenic marginal zone lymphoma with leukemic manifestation; clinical, flow cytometry, and histopathological findings of 11 cases // J. Clin. Exp. Hematop. - 2010. - Vol. 50, N 2. - P. 107-112.</mixed-citation></ref><ref id="B5"><label>5.</label><mixed-citation>Martin P., Rutherford S., Leonard J.P. Splenic lymphomas: is there still a role for splenectomy? // Oncology (Williston Park). - 2012. - Vol. 26, N 2. - P. 204-206.</mixed-citation></ref><ref id="B6"><label>6.</label><mixed-citation>Michel M. Characteristics of warm autoimmune hemolytic anemia and Evans syndrome in adults // Press Med. - 2008. - Vol. 37, N 9. - P. 1309-1318.</mixed-citation></ref><ref id="B7"><label>7.</label><mixed-citation>Piris M.A., Arribas A., Mollejo M. Marginal zone lymphoma // Semin. Diagn. Pathol. - 2011. - Vol. 28, N 2. - P. 135-145.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Thieblemont C., Davi F., Noguera M.E. et al. Splenic marginal zone lymphoma: current knowledge and future directions // Oncology (Williston Park). - 2012. - Vol. 26, N 2. - P. 194-202.</mixed-citation></ref></ref-list></back></article>
