Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya31786Editorial articleПередовая статьяResearch ArticleCardiac involvement in thrombotic microangiopathiesПоражение сердца при тромботических микроангиопатияхTuterD SТутерД СKopylovF YuКопыловФ ЮKozlovskayaN LКозловскаяН ЛDemyanovaK AДемьяноваК АShchekochikhinD YuЩекочихинД ЮShilovE MШиловЕ МSyrkinA LСыркинА Л«Первый МГМУ им. И.М. Сеченова» Минздрава России15092015879VOL 87, NO9 ()ТОМ 87, №9 (2015)172510042020Copyright ©; 2015, Consilium MedicumCopyright ©; 2015, ООО "Консилиум Медикум"2015Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/31786

Aim. To describe cardiac involvement in patients with acute thrombotic microangiopathy (TMA). Materials and methods. The case histories of 46 patients with proven TMA, including 17 patients diagnosed with atypical hemolytic uremic syndrome (aHUS) and 29 patients with catastrophic antiphospholipid syndrome (CAPS), were analyzed. Results. Different documentarily verified signs of cardiac involvement were revealed in 6 (13%) patients (5 and 1 patients diagnosed as having aHUS and CAPS, respectively). Five patients developed myocardial involvement at disease onset in the presence of multiple organ dysfunction. Conclusion. Cases of cardiac involvement in TMA of various genesis are presented. The exact incidence of myocardial involvement and its prognostic value are unknown so far.

Цель исследования. Описать поражение сердца у больных острой тромботической микроангиопатией (ТМА). Материалы и методы. Проанализировали 46 историй болезни пациентов с доказанной острой ТМА, из которых у 17 диагностирован атипичный гемолитико-уремический синдром (аГУС), у 29 — катастрофический антифосфолипидный синдром (КАФС). Результаты. Различные документально подтвержденные признаки поражения сердца выявлены у 6 (13%) больных (5 — с диагнозом аГУС, 1 — с КАФС). У 5 пациентов вовлечение миокарда развилось в дебюте заболевания в рамках полиорганного поражения. Заключение. Представлены наблюдения поражения сердца при ТМА разного генеза. Точная частота вовлечения миокарда и его прогностическое значение у пациентов с ТМА до настоящего времени неизвестны.

thrombotic microangiopathiesatypical hemolytic uremic syndromecatastrophic antiphospholipid syndromenoncoronarogenic myocardial infarctionheart failureтромботические микроангиопатииатипичный гемолитико-уремический синдромкатастрофический антифосфолипидный синдромнекоронарогенный инфаркт миокардасердечная недостаточностьKnobl P. Treatment of thrombotic microangiopathy with a focus on new treatment options. Hamostaseologie. 2013;2:149-159.Asherson R.A. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19:508-512.Patschan D, Witzke O, Dührsen U, Erbel R, Philipp T, Herget-Rosenthal S. Acute myocardial infarction in thrombotic microangiopathies — clinical characteristics, risk factors and outcome. Nephrol Dial Transplant. 2006;21:1549-1554.Демьянова К.А., Козловская Н.Л., Боброва Л.А., Кучиева А.М. Полиорганная недостаточность при острых тромботических микроангиопатиях. Нефрология и диализ. 2013;4:368.Podolsky SH, Zembowicz A, Schoen FJ, Benjamin RJ, Sonna LA. Massive myocardial necrosis in thrombotic thrombocytopenic purpura: a case report and review of the literature. Arch Pathol Laboratory Med. 1999;123(10):937-940.Zaid G, Dawod S, Rosenschein U. Immune thrombocytopenic purpura and myocardial infarction: a dilemma of management. Isr Med Assoc J. 2013;15(12):775-776.Hawkins BM, Abu-Fadel M, Vesely SK, George JN. Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review. Transfusion. 2008;48:382-392.Noris M, Remuzzi G. Cardiovascular complications in atypical haemolytic uraemic syndrome. Nat Rev Nephrol. 2014;10(3): 174-180.Филатов Л.Б. Тромботическая тромбоцитопеническая пурпура (болезнь Мошковица). Методическое пособие. Екатеринбург; 2006:42.Furlan M, Robles R, Solenthaler M, Lammle B. Acquired deficiency of von Willebrand factor — cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood. 1998;91(8):2839-2846.Levy GG, Nichols WC, Lian EC. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413(6855):488-494.Tsai HM, Lian EC. Antibodies to von Willebrand factor — cleaving protease in acute thrombotic thrombocytopenic purpura. N EnglJ Med. 1998;339(22):1585-1494.Mannucci PM. Platelet von Willebrand factor in inherited and acquired bleeding disorders. Proc Natl Acad Sci USA. 1995;92(7):2428-2432.Neild GH. Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura: pathophysiology and treatment. Kidney Int. 1998;64:45-49.Warwicker P, Goodship TH, Donne RL, Pirson Y, Nicholls A, Ward RM, Turnpenny P, Goodship JA. Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int. 1998;53:836-844.Kavanagh D, Goodship T. Genetics and complement inatypical HUS. PediatrNephrol. 2010;25:2431-2442.Constantinescu AR, Bitzan M, Weiss LS, Christen E, Kaplan BS, Cnaan A, Trachtman H. Non-enteropathic hemolytic uremic syndrome: Causes and short-term course. Am J Kidney Dis. 2004;43:976-982.Schieppati A, Ruggenenti P, Cornejo RP, Ferrario F, Gregorini G, Zucchelli P, Rossi E, Remuzzi G. Renal function at hospital admission as a prognostic factor in adult hemolytic uremic syndrome. The Italian Registry of Haemolytic Uremic Syndrome. J Am SocNephrol. 1992;2:1640-1644.Taylor CM, Chua C, Howie AJ. Clinico-pathological findings in diarrhoea-negative haemolytic uraemic syndrome. Pediatr Nephrol. 2004;19:419-425.Loirat C, Frémeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:60.Morgan HP, Schmidt CQ, Guariento M, Blaum BS, Gillespie D, Herbert AP, Kavanagh D, Mertens HD, Svergun DI, Johansson CM, Uhrin D, Barlow PN, Hannan JP. Structural basis for engagement by complement factor H of C3b on a self surface. Nat StructMol Biol. 2011;18(4):463-470.Licht C, Pluthero FG, Li L, Christensen H, Habbig S, Hoppe B, Geary DF, Zipfel PF, Kahr WH. Platelet-associated complement factor H in normal individuals and patients with atypical HUS. Blood. 2009;114:4538-4545.Stahl AL, Vaziri-Sani F, Heinen S, Kristoffersson AC, Gydell KH, Raafat R, Gutierrez A, Beringer O, Zipfel PF, Karpman D. Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation. Blood. 2008;111:5307-5315.Asherson RA, Cervera R. Catastrophic antiphospholipid syndrome. Curr Opin Hematol. 2000;7:325-329.Asherson RA, Cervera R, Piette J-C, Shoenfeld Y, Espinosa G, Petri MA, Lim E, Lau TC, Gurjal A, Jedryka-Góral A, Chwalinska-Sadowska H, Dibner RJ, Rojas-Rodríguez J, García-Carrasco M, Grandone JT, Parke AL, Barbosa P, Vasconcelos C, Ramos-Casals M, Font J, Ingelmo M. Catastrophic antiphospholipid syndrome. Clues to the pathogenesis from a series of 80 patients. Medicine. 2001;80:355-377.Cervera R, Bucciarelli S, Plasin MA, Gómez-Puerta JA, Plaza J, Pons-Estel G, Shoenfeld Y, Ingelmo M, Espinos G; Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the «CAPS Registry». J Autoimmun. 2009;32(3-4):240-245. doi:10.1016/j.jaut.2009.02.008.Asherson RA. The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment. Lupus. 1998;7:S55-S62.Ercan D, Cervera R, Asherson RA. Сatastrophic antiphospholipid syndrome. Where do we stand? Arthritis Rheum. 2003;48:3320-3327.Espinosa G, Cervera R. Antiphospholipid syndrome. Arthritis ResTher. 2008;10:230-238.Козловская Н.Л., Томилина Н.А., Столяревич Е.С., Бирюкова Л.С. и др. Катастрофический антифосфолипидный синдром у молодой больной системной красной волчанкой. Нефрология идиализ. 2010;12(3):209-221.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.Sane DC, Streer NP, Owen J. Myocardial necrosis in patients with thrombotic thrombocytopenic purpura: pathophysiology and rationale for specific therapy. Eur J Haematol. 2009;82(2):83-92.Huges C, Mc Ewan JR, Longair I. Cardiac Involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13. J Thromb Haemost. 2009;7:529-536.Hofer J, Rosales A, Fisher C, Giner T. Extra-renal manifestations of complement-mediated thrombotic microangiopathies. PediatrNephrol. 2014;2:97.Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844-1859. doi:10.2215/cjn.02210310.Sallee M, Daniel L, Piercecchi MD, Jaubert D, Fremeaux-Bacchi V, Berland Y, Burtey S.Myocardial infarction is a complication of factor H-associated atypical HUS. Nephrol Dial Transplant. 2010;25:2028-2032. doi:10.1093/ndt/gfq160.Ridolfi RL, Hutchins GM, Bell WR. The heart and cardiac conduction system in thrombotic thrombocytopenic purpura. A clinicopathologic study of 17 autopsied patients. Ann Intern Med. 1979;91:357-363.Ghandhi K, Aronow W, Desai H. Cardiovascular manifestations in patients with thrombotic thrombocytopenic purpura: a single centre experience. Clin Cardiol. 2010;33(4):213-216.Rother Rp, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: A novel mechanism of human disease. JAMA. 2005;293:1653-1652.Studt JD, Hovinga JA, Antoine G. Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS 13 inhibitor: in vitro inhibition of ADAMTS 13 activity by hemoglobin. Blood. 2005;105:542-544.Стрижаков Л.А., Козловская Н.Л., Паша С.П., Шахнова Е.А., Мирошниченко Н.Г., Метелева Н.А. Клиническое значение поражения миокарда при антифосфолипидном синдроме. Клиническая медицина. 2006;4:56-59.Bucciarelli S, Espinosa G, Cervera R. The CAPS Registry: morbidity and mortality of the catastrophic antiphospholipid syndrome. Lupus. 2009;18(10):905-912.Yamamoto H, Iwade T, Nakano R, Mohri M, Amari Y, Noma M. Numerous small vegetations revealing Libman-Sacks endocarditis in catastrophic antiphospholipid syndrome. Circulation. 2007;116:531-535.Ronco C, Haapio M, House AA, Anavekar N, Bellomo R. Cardiorenal syndrome. J Am Coll Cardiol. 2008;52(19):1527-1539.Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore). 1981;60:413-428.Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ on behalf of British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;10(1111):1365-2141.