nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1515Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESОсобенности морфологической диагностики неамилоидной формы отложения моноклональных иммуноглобулинов в почкахThe morphological diagnosis of non-amyloid form of monoclonal immunoglobulin deposition disease in the kidneyВоробьеваО. А.VorobyevaO. A.noemail@neicon.ruНастС. .NastC. .noemail@neicon.ruКоэнА. .CohenA. .noemail@neicon.ruКафедра патологической анатомии с курсом цитологии, Санкт-Петербургская медицинская академия последипломного образования, г. Санкт Петербург, РоссияRussian FederationОтдел патологии и лабораторной медицины, Медицинский центр Cedars-Sinai, Лос-Анджелес, СШАRussian Federation20072306202594421426Copyright © Воробьева О.А., Наст С..., Коэн А..., 20252025Воробьева О.А., Наст С..., Коэн А...Vorobyeva O.A., Nast C..., Cohen A...Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/1515

В настоящем исследовании изучены морфологические находки и имеющиеся на момент биопсии почки клинические и демографические данные 61 пациента с неамилоидной формой отложения моноклональных иммуноглобулинов в почках (MIDD - monoclonal immunoglobulin deposition disease), не ассоциированной с амилоидозом и/или с нефропатией Бенс-Джонса. В изучаемую группу вошли 40 случаев отложения легких цепей иммуноглобулинов, 18 случаев отложения легких и тяжелых цепей иммуноглобулинов и 3 случая отложения тяжелых цепей иммуноглобулинов. По составу депозитов парапротеина наблюдения распределились следующим образом: k (30), l (10), IgG/k (6), IgA/k (6), IgG/l (4), IgA/l (2), g (3). На световом уровне были выявлены три варианта структуры клубочков: диффузная нодулярная гломерулопатия (42,6%), диффузное расширение мезангия (27,9%) и неизмененные клубочки (29,5%). Различная степень тяжести атрофии канальцев была отмечена в 95% наблюдений. Выявление депозитов в канальцевых, гломерулярных и гладкомышечных базальных мембранах существенно отличалось при иммунофлюоресцентном (100; 91,8 и 54,1% соответственно) и электронно-микроскопическом (55,7; 45,9 и 4,9% соответственно) исследованиях. Наиболее частыми клиническими проявлениями были азотемия (68,9%) и протеинурия (55,8%). Нефротический синдром и протеинурия тяжелой степени вместе составили 27,9%.

The papers shows the morphological findings of and clinical and demographic data on 61 patients with non-amyloid form of renal monoclonal immunoglobulin deposition disease (MIDD) unassociated with AL-amyloidosis and/or Bence Jones cast nephropathy: 40 cases of light-chain deposition disease, 18 cases of light-and-heavy chain deposition disease and 3 cases of heavy-chain deposition disease. According to the composition of the paraprotein deposits, the cases were distributed as follows: k (30), l (10), IgG/k (6), IgA/k (6), IgG/l (4), IgA/l (2) and g (3). Light microscopy revealed three variants of the glomerular pattern: diffuse nodular glomerulopathy (42.6%), diffuse mesangial dilation (27.9%) and intact glomeruli (29.5%). Varying severity of tubular atrophy was noted in 95% of cases. Tubular, glomerular, and smooth muscle basement membrane deposits substantially differed in immunofluorescent (100, 91.8 и 54.1%, respectively) and ultrastructural studies (55.7, 45.9 и 4.9%, respectively). Azotemia (68.9%) and proteinuria (55.8%) were most commonly revealed. Nephrotic syndrome and heavy proteinuria together made 27.9%.

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The authors declare that there are no conflicts of interest present.