nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-973Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESСистема «ADAMTS13 – фактор фон Виллебранда – тромбоциты» у больных с тромботическими микроангиопатиями при наследственных и приобретенных тромбофилиях«ADAMTS13 – von Willebrand factor (vWF) – platelet» system in patients with thrombotic microangiopathy with hereditary and acquired thrombophiliaХафизоваЕ. Ю.KhafizovaE. Y.noemail@neicon.ruКозловскаяН. Л.KozlovskayaN. L.nkozlovskaya@yandex.ruБоброваЛ. А.BobrovaL. A.noemail@neicon.ruБобковаИ. Н.BobkovaI. N.noemail@neicon.ruВаршавскийВ. А.VarshavskyV. A.noemail@neicon.ruСтоляревичЕ. С.StoliarevichE. S.noemail@neicon.ruАвдонинВ. А.AvdoninP. V.noemail@neicon.ruЗахароваЕ. В.ZakharovaE. V.noemail@neicon.ruГОУ ВПО Первый Московский государственный медицинский университет им. И.М. СеченоваРоссияI.M Sechenov First Moscow State Medical UniversityRussian FederationФГУ «ФНЦ трансплантологии и искусственных органов им. акад. В.И. Шумакова Минздравсоцразвития РФ», г. МоскваРоссияShumakov Federal Research center of Transplantology and Artificial Organs, MoscowRussian FederationИнститут биологии развития им. Н.К. Кольцова РАНРоссияKoltzov institute of developmental biologyRussian FederationГородская клиническая больница им. С.П. Боткина, г. МоскваРоссияBotkin Moscow city hospital, MoscowRussian Federation201117062025134432438Copyright © Хафизова Е.Ю., Козловская Н.Л., Боброва Л.А., Бобкова И.Н., Варшавский В.А., Столяревич Е.С., Авдонин В.А., Захарова Е.В., 20252025Хафизова Е.Ю., Козловская Н.Л., Боброва Л.А., Бобкова И.Н., Варшавский В.А., Столяревич Е.С., Авдонин В.А., Захарова Е.В.Khafizova E.Y., Kozlovskaya N.L., Bobrova L.A., Bobkova I.N., Varshavsky V.A., Stoliarevich E.S., Avdonin P.V., Zakharova E.V.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/973

Дефицит ADAMTS13 играет ключевую роль в патогенезе тромботической тромбоцитопенической пурпуры (ТТП). В последние годы снижение активности ADAMTS13 выявляют не только при микроангиопатических синдромах, но и ряде гломерулярных болезней почек, в том числе при хроническом гломерулонефрите, волчаночном нефрите. Целью нашего исследования было оценить состояние системы «ADAMTS13 – фактор фон Виллебранда – тромбоциты» у больных с тромбофилиями, имеющих заболевания почек. Обследовано 20 больных с тромбофилиями (антифосфолипидным синдромом и генетическими) и заболеваниями почек, в том числе с волчаночным нефритом, хроническим гломерулонефритом, имевших морфологические признаки тромботической микроангиопатии (ТМА). Активность ADAMTS13 ниже крайнего референсного значения (<93%), составившая 92–56%, обнаружена у 8 (40%) больных. Активность ADAMTS13 обратно коррелировала с уровнем фактора фон Виллебранда (r = –0,3, p < 0,05) и прямо с числом тромбоцитов (r = 0,57, p < 0,01). Наиболее низкие значения активности ADAMTS13 отмечены у двух больных с гистологической картиной острой ТМА в биоптатах почек (56% и 76%). Результаты нашего исследования позволяют рассматривать нарушения в системе «ADAMTS-13 – vWF – тромбоциты» как один из универсальных патогенетических механизмов тромбообразования в сосудах микроциркуляторного русла почек при различных видах патологии. Исследование проводится при поддержке РФФИ (грант № 09-04-00468-а)

Introduction and aims: It is known that ADAMTS13 deficit is a reason of thrombotic thrombocytopenic purpura. A reduction of the ADAMTS13 activity has been recently demonstrated in different glomerular nephropathy (lupus nephritis, chronic glomerulonephritis). The aim of our study was to assess the «ADAMTS13 – von Willebrand factor – platelet» system disturbances in patients with nephropathy and thrombophilia with ТМА in biopsy specimens. We evaluated the ADAMTS13 activity in plasma of 20 patients with nephropathy and thrombophilia (antiphospholipid syndrome and hereditary thrombophilia). The ADAMTS13 activity <93% was founded in 8 (40%) pts. The lowest ADAMTS13 activity (56% and 76%) was noted in 2 patients with acute TMA. ADAMTS13 activity directly correlated with platelet counts (r = 0,5, p = 0,012) and there is indirect correlation between ADAMTS13 activity and vWF (r = –0,3, p < 0,05). Our results suggest that the disturbances in the «ADAMTS13 – von Willebrand factor – platelet» system is an universal pathogenetic mechanisms of thrombosis formation in renal microcirculation.

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