nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-803Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSТромботическая тромбоцитопеническая пурпура; случай успешного леченияThrombotic thrombocytopenic purpura; the case of successful treatmentИвановаЕ. С.IvanovaE. S.katerineiv@mail.ruТомилинаН. А.TomilinaN. A.noemail@neicon.ruПодкорытоваО. Л.PodkorytovaO. L.noemail@neicon.ruАртюхинаЛ. Ю.ArtyukhinaL. Y.noemail@neicon.ruГородская клиническая больница № 52, Москва ФГУ «ФНЦ трансплантологии и искусственных органов им. академика В.И. Шумакова» Минздравсоцразвития, Москва Кафедра нефрологии ФПДО МГМСУРоссияCity hospital № 52, Moscow Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow Moscow State University of Medicine and Dentistry, Russian Federal Health AgencyRussian Federation201217062025142114122Copyright © Иванова Е.С., Томилина Н.А., Подкорытова О.Л., Артюхина Л.Ю., 20252025Иванова Е.С., Томилина Н.А., Подкорытова О.Л., Артюхина Л.Ю.Ivanova E.S., Tomilina N.A., Podkorytova O.L., Artyukhina L.Y.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/803

Тромботическая тромбоцитопеническая пурпура (ТТП) является редким, угрожающим жизни заболеванием, которое характеризуется микроангиопатической гемолитической анемией, тромбоцитопенией, неврологическими нарушениями, почечной недостаточностью и лихорадкой. В основе патогенеза ТТП – образование в артериолах и капиллярах тромбоцитарных тромбов вследствие тяжелого дефицита специфической металлопротеазы, ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type I motif 13), расщепляющей сверхкрупные мультимеры фактора Виллебранда в плазме крови, что необходимо для предупреждения спонтанного тромбообразования в системе микроциркуляции. Прогноз ТТП значительно улучшился после введения в клиническую практику инфузий свежезамороженной плазмы или плазмообмена, которые привели к снижению летальности при этом заболевании с 90% до менее 20%. В настоящем сообщении приводится клиническое наблюдение 39-летней женщины с тяжелым течением острой ТТП, успешно леченной сеансами плазмообмена. В статье также приведены последние данные о патогенезе и лечении ТТП.

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurological and renal abnormalities and fever. The pathogenesis TTP is related to a severe deficiency of specific metalloprotease, ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type I motif 13) that cleaves the largest von Willebrand factor multimers in plasma and prevents the spontaneous formation of platelet thrombi in the microcirculation. Prognosis of TTP has been improved by plasma therapy decreasing the mortality rate from 90% to less than 20%. A case of severe acute TTP in 39-year-old woman, successfully treated with repeated plasma exchange is presented. Recent data on pathogenesis and treatment of TTP are briefly reviewed.

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