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Том 14 №2 2012 год - Нефрология и диализ

Тромботическая тромбоцитопеническая пурпура; случай успешного лечения

Иванова Е.С. Томилина Н.А. Подкорытова О.Л. Артюхина Л.Ю.

Аннотация: 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.

Для цитирования: Иванова Е.С., Томилина Н.А., Подкорытова О.Л., Артюхина Л.Ю. Тромботическая тромбоцитопеническая пурпура; случай успешного лечения. Нефрология и диализ. 2012. 14(2):114-122. doi:

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Ключевые слова: тромботическая тромбоцитопеническая пурпура, гемолитико-уремический синдром, тромботическая микроангиопатия, фактор Виллебранда, ADAMTS 13, плазмообмен, иммуносупрессивные препараты, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, thrombotic microangiopathy, von Willebrand factor, ADAMTS 13, plasma exchange, immunosuppressive agents

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