nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/2618-9801-2021-1suppl-62-73nid-20Research ArticleПРИЛОЖЕНИЕ - ВИЧ И ХБПSUPPLEMENT - HIV AND CKDТромботическая микроангиопатия у ВИЧ-инфицированных пациентов. Обзор литературы и клиническое наблюдениеThrombotic microangiopathy in HIV-infected patients. Review of literature and case reportГаджикулиеваМ. М.GadzhikulievaM. M.noemail@neicon.ruЗахароваЕ. В.ZakharovaE. V.helena.zakharova@gmail.comВолгинаГ. В.VolginaG. V.noemail@neicon.ruСтоляревичЕ. С.StolyarevichE. S.noemail@neicon.ruФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова МЗ РФ»РоссияA.I. Evdokimov Moscow State University of Medicine and DentistryRussian FederationФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова МЗ РФ»; ГБУЗ «Городская клиническая больница им. С.П. Боткина Департамента здравоохранения Москвы»РоссияA.I. Evdokimov Moscow State University of Medicine and Dentistry; S.P. Botkin City Clinical HospitalRussian FederationФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова МЗ РФ»; ГБУЗ «Городская клиническая больница № 52 Департамента здравоохранения Москвы»РоссияA.I. Evdokimov Moscow State University of Medicine and Dentistry; City Clinical Hospital No 52Russian Federation2021210620242316273Copyright © Гаджикулиева М.М., Захарова Е.В., Волгина Г.В., Столяревич Е.С., 20242024Гаджикулиева М.М., Захарова Е.В., Волгина Г.В., Столяревич Е.С.Gadzhikulieva M.M., Zakharova E.V., Volgina G.V., Stolyarevich E.S.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/20

Тромботическая микроангиопатия (ТМА) представляет собой клинико-морфологический синдром, возникающий при ряде заболеваний с различным патогенезом, и характеризующийся окклюзивным поражением сосудов микроциркуляторного русла, тромбоцитопенией, микроангиопатическим гемолизом и ишемическими органными повреждениями. В настоящее время выделяют две первичные формы ТМА - тромботическую тромбоцитопеническую пурпуру (ТТП) и гемолитико-уремический синдром (ГУС). Многочисленные вторичные формы ТМА могут быть ассоциированы с беременностью, трансплантацией, инфекциями, аутоиммунными заболеваниями, злокачественными новообразованиями, лекарственной терапией и рядом других заболеваний или состояний. Ключевым звеном патогенеза ТТП является снижение активности металлопротеазы ADAMTS-13, клинически классическая триада ТТП представляет сочетание микроангиопатического гемолиза, тяжелой тромбоцитопении и поражения ЦНС. ТТП подразделяют на врожденную и иммуноопосредованную, которая, в свою очередь, подразделяется на первичную, при которой невозможно установить причину или состояние, запустившее продукцию антител, и вторичную, ассоциированную с другими заболеваниями или факторами. К ним относятся аутоиммунные заболевания, лекарственные препараты, беременность, злокачественные новообразования и инфекции. Развитие ТМА при ВИЧ-инфекции было впервые описано в 1984 году, с этого времени опубликовано значительное число наблюдений, и, хотя ТМА является достаточно редким осложнением ВИЧ-инфекции, на сегодняшний день существование ассоциации между этими состояниями не вызывает сомнений. Повреждение эндотелия, которое, по-видимому, является причиной активации тромбоцитов и тромбозов микроциркуляторного русла, при ВИЧ-инфекции может быть опосредовано прямой инвазией вируса в эндотелиальные клетки или эффектом цитокинов, либо действием связанных с ВИЧ белков. По имеющимся данным ВИЧ-инфекция увеличивает риск развития ТТП в 15-40 раз по сравнению с общей популяцией, особенно у лиц с поздними стадиями заболевания, а частота ВИЧ-ассоциированного ГУС даже превышает частоту ТТП. Независимо от формы ТМА, развитие этого осложнения у ВИЧ-инфицированных лиц ассоциировано почти с 6-кратным увеличением летальности. И, хотя у большинства пациентов с ВИЧ-ассоциированной ТМА имеется тяжелая стадия ВИЧ-инфекции, в 28% случаев именно ТМА может оказаться первым проявлением болезни. Мы представляем клиническое наблюдение пациента с иммуноопосредованной ТТП, ассоциированной с ВИЧ-инфекцией. Особенность представленного случая состоит в том, что диагноз ТТП был установлен при жизни и подтвержден посмертно, а диагноз ВИЧ-инфекции - установлен лишь посмертно.

Thrombotic microangiopathy (TMA) is a syndrome representing a broad range of diseases and conditions, and characterized by microvasculature occlusion, thrombocytopenia, microangiopathic hemolytic anemia, and ischemic organ damage. Currently, classification distinguishes two primary forms of TMA - thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS). Various secondary TMA forms are associated with pregnancy, organ transplantation, infections, autoimmune diseases, malignancies, drug exposure, and other conditions. The key feature of TTP pathogenesis is decreased activity of ADAMTS-13 metalloprotease, the classical clinical presentation is a tirade of microangiopathic hemolytic anemia, severe thrombocytopenia, and central nervous system damage. TTP may be innate or immune-mediated; the latter may be idiopathic or associated with underlying conditions, again like autoimmune diseases, drugs, pregnancy, malignancies, and infections. TMA associated with HIV-infection was described in 1984, and since that time, many cases and case series had been reported. Even though TMA is a relatively rare complication of HIV-infection, nowadays association between these conditions is well established. Endothelial damage, which is now considered a primary cause of platelet activation and microvasculature thrombosis, in HIV-infection may be a consequence of the direct viral invasion, or indirectly associated with cytokines and HIV-related proteins. HIV poses a risk of TTP 15-40 times higher compared to the non-infected persons, and the incidence of HIV-associated HUS is even higher. However, regardless of its form, TMA in HIV-infected patients known to be associated with 6-fold increased mortality. Although the majority of patients with HIV-associated TMA present with late stages of severe HIV-infection, in 28% of cases TMA turns to be the first presentation of the disease. We present here a case of immune-mediated TTP, associated with HIV-infection. In this particular case diagnosis of TTP was established on a clinical basis, however, its confirmation by almost zero ADAMTS-13 activity arrived post-mortem, while HIV-infection was discovered post-mortem only and confirmed by immune blotting.

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