Обсуждаются подходы к лечению приобретенной тромботической тромбоцитопенической пурпуры (ТТП). Плазмообмены, глюкокортикостероидные гормоны позволяют купировать острый приступ ТТП, а применение ритуксимаба – достигнуть ремиссии. В последние годы используется каплацизумаб, обсуждается его место в лечении ТТП. Описано также применение циклоспорина А, бортезомиба, спленэктомии, N-ацетилцистеина, рекомбинантного ADAMTS13, сопутствующей терапии. Обсуждаются вопросы ведения пациенток с ТТП во время беременности, лечения ТТП у детей.
The review discusses approaches to treatment of acquired thrombotic thrombocytopenic purpuгa (aTTP). In patients with aTTP plasma exchanges, glucocorticosteroids allow to stop an acute attack of TTP, and use of rituximab allows to achieve remission. In recent years, caplacizumab has been used. Treatment options such as cyclosporin A, bortezomib, splenectomy, N-acetylcysteine, recombinant ADAMTS13 are also described. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.
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1. Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15(2):312-22. DOI:10.1111/jth.13571
2. Yamada Y, Ohbe H, Yasunaga H, et al. Clinical Practice Pattern of Acquired Thrombotic Thrombocytopenic Purpura in Japan: A nationwide Inpatient Database Analysis. Blood. 2019;134(Suppl. 1):2374. DOI:10.1182/blood-2019-125170
3. Picod A, Coppo P. When targeted therapies alleviate the burden of TPE: The example of immune-mediated TTP. Transf Apher Sci. 2019;58(3):273-7. DOI:10.1016/j.transci.2019.04.012
4. Nguyen TC, Han YY. Plasma exchange therapy for thrombotic microangiopathies. Organogenesis. 2011;7(1):28-31. DOI:10.4161/org.7.1.14027
5. Rock GA, Shumak KH, Buskard NA, et al. Comparison of Plasma Exchange with Plasma Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura. N Engl J Med. 1991;325(6):393-7. DOI:10.1056/NEJM199108083250604
6. Henon P. Thrombotic thrombocytopenic purpura: Clinical results of a French controlled trial. Transfus Sci. 1992;13(1):63-72. DOI:10.1016/0955-3886(92)90123-X
7. Von Baeyer H. Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies. Ther Apher. 2002;6(4):320-8. DOI:10.1046/j.1526-0968.2002.00390.x
8. Rock G, Shumak KH, Sutton DMC, et al. Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura. Brit J Haematol. 1996;94(2):383-6. DOI:10.1046/j.1365-2141.1996.d01-1800.x
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1 ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, Москва, Россия;
2 ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, Москва, Россия
*gengalst@gmail.com
________________________________________________
Gennadii M. Galstyan*1, Alexei A. Maschan2, Elizaveta E. Klebanova1, Irina I. Kalinina2
1 National Research Center for Hematology, Moscow, Russia;
2 Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
*gengalst@gmail.com