bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.18821/0234-5730-2017-62-2-96-100bloodjour-69Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSОБМЕННЫЙ ПЛАЗМАФЕРЕЗ В ЛЕЧЕНИИ ТРОМБОТИЧЕСКОЙ ТРОМБОЦИТОПЕНИЧЕСКОЙ ПУРПУРЫTHE PLASMA EXCHANGE IN THE THEATMENT OF THROMBOTIC THROMBOCYTOPENIC PURPURAhttps://orcid.org/0000-0001-6010-9583ФоминA. М.FominA. M.

129110, г. Москва

Фомин Александр Михайлович, доктор мед. наук, профессор, руководитель Отделения хирургической гемокоррекции и детоксикации

129110, Moscow

Fomin Aleksandr M., MD, PhD

amf05@mail.ruhttps://orcid.org/0000-0002-3291-6350ЗацепинаA. A.ZatsepinaA. A.

129110, г. Москва

129110, Moscow

https://orcid.org/0000-0002-6523-9157ГоленковA. К.GolenkovA. K.

129110, г. Москва

129110, Moscow

https://orcid.org/0000-0003-2847-4374ЗахаровС. Г.ZakharovS. G.

129110, г. Москва

129110, Moscow

https://orcid.org/0000-0003-2650-7646КатаеваE. В.KataevaE. V.

129110, г. Москва

129110, Moscow

ГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского»РоссияMoscow Regional Clinical and Research InstituteRussian Federation20171203201962296100Copyright © Фомин A.М., Зацепина A.A., Голенков A.К., Захаров С.Г., Катаева E.В., 20192019Фомин A.М., Зацепина A.A., Голенков A.К., Захаров С.Г., Катаева E.В.Fomin A.M., Zatsepina A.A., Golenkov A.K., Zakharov S.G., Kataeva 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://www.htjournal.ru/jour/article/view/69

Представлены клинические случаи лечения тромботической тромбоцитопенической пурпуры с использованием многократных обменных плазмаферезов. Описаны сложности быстрой диагностики и значение раннего начала патогенетически детерминированной терапии. Обсуждена тактика использования экстракорпоральной гемокоррекции в терапии данного заболевания.

Clinical observations of plasma exchange therapy in patients with thrombotic thrombocytopenic purpura (TTP) are presented. Difficulties in the early diagnosis and the role of the early use of the pathogenic treatment are considered. The impact of extracorporal therapy in patients with TTP is discussed.

тромботическая тромбоцитопеническая пурпураболезнь МошковицаплазмаферезADAMTS-13гемолитическая анемияthrombotic thrombocytopenic purpuraMoschcowitz diseaseplasma exchange therapyADAMTS-13hemolytic anemiaReferencesMoschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries, an undescribed disease. Arch. Intern. Med. 1925; 36: 89–93.Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries, an undescribed disease. Arch. Intern. Med. 1925; 36: 89–93.Войцеховский В.В., Филатов Л. Б., Пивник А.В., Авдонин П.В., Есенина Т.В., Судаков А.Г. Особенности диагностики и лечения тромботической тромбоцитопенической пурпуры, развившейся во время беременности: обзор литературы и собственное наблюдение. Клиническая онкогематология. Фундаментальные исследования и клиническая практика. 2014; 4(7): 587–98.Voytsekhovskiy V.V., Filatov L.B., Pivnik A.V., Avdonin P.V., Esenina T.V., Sudakov A.G. Diagnosis and treatment of thrombotic thrombocytopenic purpura developed during gestation: literature review and case report. Clinical Oncohematology. Basic Research and Clinical Practice. Russian Journal (Klinicheskaya onkogematologiya. Fundamentalnye issledovaniya i klinicheskaya praktika). 2014; 4(7): 587–98. (in Russian)Филатов Л.Б. Тромботические микроангиапатии. Клиническая онкогематология. Фундаментальные исследования и клиническая практика. 2008; 1(4): 366–76.Filatov L.B. Thrombotic microangiopathies. Clinical Oncohematology. Basic Research and Clinical Practice. Russian Journal (Klinicheskaya onkogematologiya. Fundamentalnye issledovaniya i klinicheskaya praktika). 2008; 1(4): 366–76. (in Russian)Scully M., Hunt B.J., Benjamin S., Liesner R., Rose P., Peyvandi F., et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br. J. Haematol. 2012; 158(3): 323–35.Scully M., Hunt B.J., Benjamin S., Liesner R., Rose P., Peyvandi F., et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br. J. Haematol. 2012; 158(3): 323–35.Singer K., Bornstein F.P., Wile S.A. Thrombotic thrombocytopenic purpura: hemorrhagic diathesis with generalized platelet thromboses. Blood. 1947; 2(6): 542–54.Singer K., Bornstein F.P., Wile S.A. Thrombotic thrombocytopenic purpura: hemorrhagic diathesis with generalized platelet thromboses. Blood. 1947; 2(6): 542–54.Noris M., Remuzzi G. Genetics and genetic testing in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. Semin. Nephrol. 2010; 30(4): 395–408. doi: 10.1016/j.semnephrol.2010.06.006.Noris M., Remuzzi G. Genetics and genetic testing in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. Semin. Nephrol. 2010; 30(4): 395–408. doi: 10.1016/j.semnephrol.2010.06.006.George J.N., Vesely S.K., Terrell D.R., Deford C.C., Reese J.A., AlNouri Z.L., et al. The Oklahoma Thrombotic Thrombocytopenic Purpura – haemolytic Uraemic Syndrome Registry. A model for clinical research, education and patient care. Hamostaseologie. 2013; 33(2): 105–12. doi: 10.5482/HAMO-12-10-0016.George J.N., Vesely S.K., Terrell D.R., Deford C.C., Reese J.A., AlNouri Z.L., et al. The Oklahoma Thrombotic Thrombocytopenic Purpura – haemolytic Uraemic Syndrome Registry. A model for clinical research, education and patient care. Hamostaseologie. 2013; 33(2): 105–12. doi: 10.5482/HAMO-12-10-0016.Amorosi E.L., Ultmann J.E. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore). 1966; 45(2): 39–59.Amorosi E.L., Ultmann J.E. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore). 1966; 45(2): 39–59.Bell W.R., Braine H.G., Ness P.M., Kickler T.S. Improved survival in thrombotic thrombocytopenic purpura – hemolytic uremic syndrome. Clinical experience in 108 patients. N. Engl. J. Med. 1991; 325(6): 398–403.Bell W.R., Braine H.G., Ness P.M., Kickler T.S. Improved survival in thrombotic thrombocytopenic purpura – hemolytic uremic syndrome. Clinical experience in 108 patients. N. Engl. J. Med. 1991; 325(6): 398–403.Иванова Е.С. Томилина Н.А. Подкорытова О.Л. Артюхина Л.Ю. Тромботическая тромбоцитопеническая пурпура: случай успешного лечения. Нефрология и диализ. 2012; 14(2): 114–22.Ivanova E.S., Tomilina N.A., Podkorytova O.L., Artyukhina L.Y. Thrombotic thrombocytopenic purpura; the case of successful treatment. Nephrology and dialysis. Russian Journal (Nephrologiya i dialis). 2012; 14(2): 114–22. (in Russian)Чеснокова Н.П., Невважай Т.А., Моррисон В.В., Бизенкова М.Н. Лекция 6. Приобретенные гемолитические анемии. Этиология и патогенез, гематологическая характеристика. Международный журнал прикладных и фундаментальных исследований. 2015; 6-1: 167–71.Chesnokova N.P., Nevvazhay T.A., Morrison V.V., Bizenkova M.N. Lecture 6. Acquired hemolytic anemia. Etiology and pathogenesis, hematological characteristic. International journal of applied and fundamental research. Russian Journal (Mezhdunarodnyy zhurnal prikladnykh i fundamentalnykh issledovaniy). 2015; 6-1: 167–71. (in Russian)Allford S.L., Hunt B.J., Rose P., Machin S. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br. J. Haematol. 2003; 120 (4): 556–73.Allford S.L., Hunt B.J., Rose P., Machin S. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br. J. Haematol. 2003; 120 (4): 556–73.George J.N. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010; 116(20): 4060–9. doi: 10.1182/blood-2010-07-271445.George J.N. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010; 116(20): 4060–9. doi: 10.1182/blood-2010-07-271445.Tsai H.M. Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update. Hematol. Oncol. Clin. N. Am. 2013; 27(3): 565–84.Tsai H.M. Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update. Hematol. Oncol. Clin. N. Am. 2013; 27(3): 565–84.Zhan H., Streiff M.B., King K.E., Segal J.B. Thrombotic thrombocytopenic purpura at the Johns Hopkins Hospital from 1992 to 2008: clinical outcomes and risk factors for relapse. Transfusion. 2010; 50(4): 868–74.Zhan H., Streiff M.B., King K.E., Segal J.B. Thrombotic thrombocytopenic purpura at the Johns Hopkins Hospital from 1992 to 2008: clinical outcomes and risk factors for relapse. Transfusion. 2010; 50(4): 868–74.Калинина И.И., Сталина О.В., Рыжко В.В., Цветаева Н.В., Васильев С.А., Петрова В.И. Болезнь Мошковиц, хроническое рецидивирующее течение. Терапевтический архив. 2008; 80(7): 65–7.Kalinina I.I., Stalina O.V., Ryzhko V.V., Tsvetaeva N.V., Vasiliev S.A., Petrova V.I. A chronic recurrent course of Moschcowitz disease. Therapeutic archive. Russian Journal (Terapevticheskiy arkhiv). 2008; 80(7): 65–7. (in Russian)Furlan M., Robles R., Galbusera M., Remuzzi G., Kyrle P., Brenner B., et al. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N. Engl. J. Med. 1998; 339(22): 1578–84.Furlan M., Robles R., Galbusera M., Remuzzi G., Kyrle P., Brenner B., et al. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N. Engl. J. Med. 1998; 339(22): 1578–84.Furlan M., Robles R., Lаmmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996; 87(10): 4223–34.Furlan M., Robles R., Lаmmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996; 87(10): 4223–34.Tsai H.M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996; 87(10): 4235–44.Tsai H.M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996; 87(10): 4235–44.Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N. Engl. J. Med. 1998; 339(22): 1585–94.Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N. Engl. J. Med. 1998; 339(22): 1585–94.Bianchi V., Robles R., Alberio L., Furlan M., Lammle B. Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002; 100(2): 710–3.Bianchi V., Robles R., Alberio L., Furlan M., Lammle B. Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002; 100(2): 710–3.Fujikawa K., Suzuki H., McMullen B., Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001; 98(6): 1662–6.Fujikawa K., Suzuki H., McMullen B., Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001; 98(6): 1662–6.Lotta L.A. Pathophysiology of Thrombotic Thrombocytopenic Purpura: the “Two-Hit” paradigm. Leiden: Leiden University Press; 2012.Lotta L.A. Pathophysiology of Thrombotic Thrombocytopenic Purpura: the “Two-Hit” paradigm. Leiden: Leiden University Press; 2012.Zheng X., Chung D., Takayama T.K., Majerus E.M., Sadler J.E., Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J. Biol. Chem. 2001; 276(44): 41059–63.Zheng X., Chung D., Takayama T.K., Majerus E.M., Sadler J.E., Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J. Biol. Chem. 2001; 276(44): 41059–63.Zheng X., Majerus E.M., Sadler J.E. ADAMTS13 and TTP. Curr. Opin. Hematol. 2002; 9(5): 389–94.Zheng X., Majerus E.M., Sadler J.E. ADAMTS13 and TTP. Curr. Opin. Hematol. 2002; 9(5): 389–94.Wyrick-Glatzel J. Thrombotic thrombocytopenic purpura and ADAMTS-13: New insights into pathogenesis, diagnosis, and therapy. Lab. Med. 2004; 35(12): 733–40.Wyrick-Glatzel J. Thrombotic thrombocytopenic purpura and ADAMTS-13: New insights into pathogenesis, diagnosis, and therapy. Lab. Med. 2004; 35(12): 733–40.George J.N., Terrell D.R., Swisher K.K., Vesely S.K. Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura – hemolytic uremic syndrome registry. J. Clin. Apher. 2008; 23(4): 129–37. doi: 10.1002/jca.20169.George J.N., Terrell D.R., Swisher K.K., Vesely S.K. Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura – hemolytic uremic syndrome registry. J. Clin. Apher. 2008; 23(4): 129–37. doi: 10.1002/jca.20169.Studt J.D., Kremer Hovinga J.A., Alberio L., Bianchi V., Lämmle B. Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory. Swiss. Med. Wkly. 2003; 133(23–24): 325–32.Studt J.D., Kremer Hovinga J.A., Alberio L., Bianchi V., Lämmle B. Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory. Swiss. Med. Wkly. 2003; 133(23–24): 325–32.Sarode R., Bandarenko N., Brecher M.E., Kiss J.E., Marques M.B., Szczepiorkowski Z.M., et al. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J. Clin. Apher. 2014; 29(3):148–67.Sarode R., Bandarenko N., Brecher M.E., Kiss J.E., Marques M.B., Szczepiorkowski Z.M., et al. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J. Clin. Apher. 2014; 29(3):148–67.Coppo P., Bussel A., Charrier S., Adrie C., Galicier L., Boulanger E., et al. High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Medicine (Baltimore). 2003; 82(1): 27–38.Coppo P., Bussel A., Charrier S., Adrie C., Galicier L., Boulanger E., et al. High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Medicine (Baltimore). 2003; 82(1): 27–38.Nguyen L., Terrell D.R., Duvall D., Vesely S.K., George J.N. Complications of plasma exchange in patients treated for thrombotic thrombocytopenic purpura: An additional study of 43 consecutive patients, 2005–2008. Transfusion. 2009; 49(2): 392–4.Nguyen L., Terrell D.R., Duvall D., Vesely S.K., George J.N. Complications of plasma exchange in patients treated for thrombotic thrombocytopenic purpura: An additional study of 43 consecutive patients, 2005–2008. Transfusion. 2009; 49(2): 392–4.Smith J.W., Weinstein R., Hillyer K.L. Therapeutic apheresis: a summary of current indication categories endorsed by the AABB and American Society for Apheresis. Transfusion. 2003; 43(6): 820–2.Smith J.W., Weinstein R., Hillyer K.L. Therapeutic apheresis: a summary of current indication categories endorsed by the AABB and American Society for Apheresis. Transfusion. 2003; 43(6): 820–2.Ziman A., Mitri M., Klapper E., Pepkowitz S.H., Goldfinger D. Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution’s experience and review of the literature. Transfusion. 2005; 45(1): 41–9.Ziman A., Mitri M., Klapper E., Pepkowitz S.H., Goldfinger D. Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution’s experience and review of the literature. Transfusion. 2005; 45(1): 41–9.Scully M., Yarranton H. Liesner R., Cavenagh J., Hunt B., Benjamin S., et al. Regional UK TTP Registry: correlation with laboratory ADAMTS 13 analysis and clinical features. Br. J. Haematol. 2008; 142(5): 819–26.Scully M., Yarranton H. Liesner R., Cavenagh J., Hunt B., Benjamin S., et al. Regional UK TTP Registry: correlation with laboratory ADAMTS 13 analysis and clinical features. Br. J. Haematol. 2008; 142(5): 819–26.Kremer Hovinga J.A., Vesely S.K., Terrell D.R., Lämmle B., George J.N. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010; 115(8): 1500–11. doi: 10.1182/blood-2009-09-243790.Kremer Hovinga J.A., Vesely S.K., Terrell D.R., Lämmle B., George J.N. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010; 115(8): 1500–11. doi: 10.1182/blood-2009-09-243790.

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