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<article article-type="research-article" dtd-version="1.3" 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" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">nid</journal-id><journal-title-group><journal-title xml:lang="ru">Нефрология и диализ</journal-title><trans-title-group xml:lang="en"><trans-title>Nephrology and Dialysis</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1680-4422</issn><issn pub-type="epub">2618-9801</issn><publisher><publisher-name>Российское диализное общество</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.28996/2618-9801-2024-1-55-70</article-id><article-id custom-type="elpub" pub-id-type="custom">nid-144</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group></article-categories><title-group><article-title>Моноклональные гаммапатии почечного значения и парапротеинемические поражения почек при злокачественных лимфопролиферативных заболеваниях - клинико-морфологическая характеристика 197 пациентов одного центра</article-title><trans-title-group xml:lang="en"><trans-title>Clinical and pathology characteristics of 197 patients with monoclonal gammopathies of renal significance and paraprotein-related kidney damage in malignant lymphoproliferative disorders</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Захарова</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zakharova</surname><given-names>E. V.</given-names></name></name-alternatives><email xlink:type="simple">helena.zakharova@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зыкова</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Zykova</surname><given-names>A. S.</given-names></name></name-alternatives><email xlink:type="simple">ansezy@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Макарова</surname><given-names>Т. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Makarova</surname><given-names>T. A.</given-names></name></name-alternatives><email xlink:type="simple">tmakarova24@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Леонова</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Leonova</surname><given-names>E. S.</given-names></name></name-alternatives><email xlink:type="simple">janeleonova999@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Никитина</surname><given-names>Е. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Nikitina</surname><given-names>E. N.</given-names></name></name-alternatives><email xlink:type="simple">md.nikitina@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Столяревич</surname><given-names>Е. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Stolyarevich</surname><given-names>E. S.</given-names></name></name-alternatives><email xlink:type="simple">Stolyarevich@yandex.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Воробьева</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Vorobieva</surname><given-names>O. A.</given-names></name></name-alternatives><email xlink:type="simple">olvorob70@gmail.com</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шутов</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shutov</surname><given-names>E. V.</given-names></name></name-alternatives><email xlink:type="simple">shutov_e_v@mail.ru</email><xref ref-type="aff" rid="aff-6"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ГБУЗ “Городская клиническая больница имени С.П. Боткина” Департамента здравоохранения города Москвы; ФГБОУ ДПО “Российская медицинская академия непрерывного профессионального образования” Министерства здравоохранения Российской Федерации; ФГБОУ ВО «Московский государственный медико-стоматологический университет имени А.И. Евдокимова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare; Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation; A. I. Evdokimov Moscow State University of Medicine and Dentistry</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ГБУЗ “Городская клиническая больница имени С.П. Боткина” Департамента здравоохранения города Москвы; ФФМ ФГБОУ ВО «Московский Государственный Университет имени М.В. Ломоносова»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ГБУЗ “Городская клиническая больница имени С.П. Боткина” Департамента здравоохранения города Москвы</institution><country>Россия</country></aff><aff xml:lang="en"><institution>S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ФГБОУ ВО «Московский государственный медико-стоматологический университет имени А.И. Евдокимова» Министерства здравоохранения Российской Федерации; БУЗ «Городская клиническая больница №52» Департамента здравоохранения города Москвы</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A. I. Evdokimov Moscow State University of Medicine and Dentistry; City Clinical Hospital №52, Moscow City Department of Healthcare</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ООО Национальный Центр Клинической Морфологической диагностики</institution><country>Россия</country></aff><aff xml:lang="en"><institution>National Center of Clinical Morphological Diagnostics</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>ГБУЗ “Городская клиническая больница имени С.П. Боткина” Департамента здравоохранения города Москвы; ФГБОУ ДПО “Российская медицинская академия непрерывного профессионального образования” Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare; Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>21</day><month>06</month><year>2024</year></pub-date><volume>26</volume><issue>1</issue><fpage>55</fpage><lpage>70</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Захарова Е.В., Зыкова А.С., Макарова Т.А., Леонова Е.С., Никитина Е.Н., Столяревич Е.С., Воробьева О.А., Шутов Е.В., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Захарова Е.В., Зыкова А.С., Макарова Т.А., Леонова Е.С., Никитина Е.Н., Столяревич Е.С., Воробьева О.А., Шутов Е.В.</copyright-holder><copyright-holder xml:lang="en">Zakharova E.V., Zykova A.S., Makarova T.A., Leonova E.S., Nikitina E.N., Stolyarevich E.S., Vorobieva O.A., Shutov E.V.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://journal.nephro.ru/jour/article/view/144">https://journal.nephro.ru/jour/article/view/144</self-uri><abstract><p>Обоснование. Морфологические варианты ассоциированных с парапротеинами поражений почек при лимфопролиферативных заболеваниях (ЗЛПЗ) в значительной мере совпадают со спектром моноклональных гаммапатий почечного значения (МГПЗ). Целью нашего ретроспективного исследования явилось проведение сравнительного анализа клинической симптоматики и морфологических особенностей МГПЗ и ассоциированного с парапротеинемией поражения почек при ЗЛПЗ. Материалы и методы. В группу исследования вошли 197 пациентов с морфологически верифицированным парапротеинемическим поражением почек, наблюдавшихся в нефрологическом отделении ГБУЗ ГКБ им. С.П. Боткина за период с 1997 по 2022 год. Средний возраст пациентов составил 59,41±10,44 лет; соотношение мужчин и женщин 47,7/52,8%. Для дальнейшего анализа пациенты были разделены на две подгруппы, в подгруппу МГПЗ вошли 168 (85,2%) случаев, в подгруппу ЗЛПЗ - 29 (14,7%) случаев. Результаты. В подгруппе МГПЗ значимо чаще по сравнению с ЗЛПЗ наблюдались общая слабость, одышка, артериальная гипотензия, нефротический синдром, и присутствовали моноклональные легкие цепи лямбда (p=0,011, p=0,009, p=0,010, p=0,011, и р=0,015 соответственно); в подгруппе ЗЛПЗ значимо чаще присутствовали артериальная гипертензия, моноклональные легкие цепи каппа и плазматизация костного мозга. (p=0,048, р=0,003 и p&lt;0,001 соответственно). В подгруппе МГПЗ преобладающим морфологическим вариантом явился AL амилоидоз, составивший 72,6% случаев, в подгруппе ЗЛПЗ он был найден в 31,0% случаев. Болезнь отложения моноклональных иммуноглобулинов чаще наблюдалась при ЗЛПЗ, чем в рамках МГПЗ (24,1% vs 11,3% соответственно), но и в подгруппе ЗЛПЗ она уступала по частоте AL амилоидозу (24,1% vs 31,0% соответственно). Как цилиндровая нефропатия, так и проксимальная тубулопатия легких цепей, обнаружены не только при множественной миеломе, но и в рамках МГПЗ, однако выявлялись при ЗЛПЗ более чем в 10 раз чаще (13,7% vs 1,1% и 10,3% vs 0,5% соответственно). Пролиферативный гломерулонефрит с депозитами моноклональных иммуноглобулинов, атипичная мембранозная нефропатия, криоглобулинемический гломерулонефрит и C3 гломерулонефрит обнаружены только в подгруппе МГПЗ, а гломерулонефрит с антителами к гломерулярной базальной мембране - только при ЗЛПЗ. Непролиферативный гломерулонефрит с депозитами моноклональных иммуноглобулинов М выявлен в единичных случаях в обеих подгруппах, а сочетание двух вариантов повреждения при ЗЛПЗ имело место в 2,5 раза чаще чем при МГПЗ (13,7% vs 5,3% соответственно). Выводы. Независимо от злокачественной или незлокачественной природы секретирующего клона самым частым морфологическим вариантом парапротеин-ассоциированных поражений почек является AL амилоидоз, обусловленный продукцией моноклональных легких цепей лямбда, и клинически характеризующийся слабостью, одышкой, артериальной гипотензией и нефротическим синдромом; в рамках МГПЗ этот вариант наблюдается в 2,5 раза чаще, чем при ЗЛПЗ. Цилиндровая нефропатия, традиционно считавшаяся «визитной карточкой» множественной миеломы, может развиваться и при тлеющей миеломе и, тем самым, входит в спектр МГПЗ.</p></abstract><trans-abstract xml:lang="en"><p>Background. Paraprotein-related kidney damage in B-cell malignancies (BCM) often share common features with monoclonal gammopathy of renal significance (MGRS). In this single-center retrospective study, we aimed to analyze clinical presentation and pathology patterns of paraprotein-related kidney damage in malignant and non-malignant B-cell disorders. Materials and methods. Study group included 197 patients (M/F ratio 47.7/52.8%, mean age 59.41±10.44 years), with pathology-proven paraprotein-related kidney disease, followed in 1997-2022. For the further analysis, we split the patients into two sub-groups; MGRS sub-group consisted of 168 (85.2%) cases, and BCM sub-group included 29 (14.7%) cases. Results. In the MGRS sub-group general weakness, dyspnea, arterial hypotension, nephrotic syndrome and presence of monoclonal light chains lambda were found significantly more often compared to the BCM sub-group (p=0.011, p=0.009, p=0.010, p=0.011, and р=0.015 respectively). In the BCM sub-group significantly more often were arterial hypertension, presence of monoclonal light chains kappa and bone marrow plasmacytosis (p=0.048, р=0.003 and p&lt;0.001 respectively). AL amyloidosis apparently dominated in the MGRS sub-group, constituting 72.6% of cases; in the BCM sub-group it was found in 31.0% of cases. Monoclonal immunoglobulin deposition disease was the more often finding in the BCM sub-group compared to MGRS (24.1% vs 11.3% respectively), however also yielding AL amyloidosis (24.1% vs 31.0% respectively). Cast-nephropathy, as well as light chain proximal tubulopathy, presented not exclusively in multiple myeloma, but also in the MGRS setting, but were both more than ten times often in BCM (13.7% vs 1.1% and 10.3% vs 0.5% respectively). Proliferative glomerulonephritis with monoclonal immunoglobulin deposits, atypical membranous nephropathy, cryoglobulinemic glomerulonephritis and C3 glomerulonephritis were found in MGRS sub-group only; and glomerulonephritis with antibodies to glomerular basement membrane. Non-proliferative glomerulonephritis with immunoglobulin M monoclonal deposits presented in the single cases in both sub-groups. Combination of two patterns of damage presented in BCM more than twice often, compared to MGRS (13.7% vs 5.3% respectively). Conclusions. AL amyloidosis, associated with monoclonal light chains lambda and presenting with general weakness, dyspnea, arterial hypotension and nephrotic syndrome is the most often pattern of paraprotein-related kidney damage, regardless malignant or non-malignant nature of the secreting clone; however, it is much more typical for MGRS than for BCM. Cast-nephropathy, traditionally regarded as a hallmark of multiple myeloma, also seen in smoldering myeloma, thus representing MGRS.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>амилоидоз</kwd><kwd>биопсия почки</kwd><kwd>моноклональные иммуноглобулины</kwd><kwd>нефротический синдром</kwd><kwd>почечная недостаточность</kwd><kwd>хроническая болезнь почек</kwd><kwd>amyloidosis</kwd><kwd>chronic kidney disease</kwd><kwd>kidney biopsy</kwd><kwd>kidney failure</kwd><kwd>monoclonal immunoglobulin</kwd><kwd>nephrotic syndrome</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Mousavi S.E., Ilaghi M., Aslani A., et al. A population-based study on incidence trends of myeloma in the United States over 2000-2020. Sci Rep. 2023. 13: 20705. doi: 10.1038/s41598-023-47906-y.</mixed-citation><mixed-citation xml:lang="en">Mousavi S.E., Ilaghi M., Aslani A., et al. 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