Клиническое значение определения металлопротеиназы ADAMTS-13, ее ингибитора и фактора фон Виллебранда при патологических состояниях в неонатальном периоде
Проведен систематический анализ данных о роли металлопротеиназы ADAMTS-13 в патогенезе различных патологических состояний неонатального периода. В обзор включены данные зарубежных и отечественных статей, найденных в e-Library и PubMed по данной теме, опубликованных за последние 10 лет. Представлены данные по диагностическим критериям, возможностям определения ADAMTS-13 и ее ингибиторов, значения ADAMTS-13 в развитии тромботической тромбоцитопенической пурпуры и неонатальных тромбозов. Изложены современные представления о возможностях лечения и коррекции вышеуказанных состоянийБицадзе В.О., Григорьева К.Н., Илалами И., Макацария А.Д., Мингалимов М.А., Воробьев А.В., Хизроева Д.Х., Мэн М.
Ключевые слова
ADAMTS-13
тромботическая тромбоцитопеническая пурпура
неонатальные тромбозы
vWF
нарушения гемостаза
тромбофилия
антикоагулянтная терапия
Список литературы
- Nowak-Gottl U., von Kries R., Gobel U. Neonatal symptomatic thromboembolism in Germany: two year survey. Arch. Dis. Child. Fetal Neonatal Ed. 1997; 76(3): F163-7. DOI: 10.1136/fn.76.3.f163
- Chalmers E.A. Neonatal thrombosis. J. Clin. Pathol. 2000; 53(6): 419-23. http://dx.doi.org/10.1136/jcp.53.6.419
- Veldman A., Nold M.F., Michel-Behnke I. Thrombosis in the critically ill neonate: incidence, diagnosis, and management. Vasc. Health Risk Manag. 2008; 4(6): 1337-48. DOI: 10.2147/vhrm.s4274
- Moake J.L., Rudy C.K., Troll J.H., Weinstein M.J., Colannino N.M., Azocar J., et al. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N. Engl. J. Med. 1982; 307(23): 1432-5. DOI: 10.1056/NEJM198212023072306
- Springer T. Von Willebrand factor, Jedi knight of the bloodstream. Blood. 2014; 124(9): 1412-25. doi: 10.1182/blood-2014-05-378638.
- van Schooten C.J., Shahbazi S., Groot E., Oortwijn B.D., van den Berg H.M., Denis C.V., Lenting P.J. Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo. Blood. 2008; 112(5): 1704-12. doi: 10.1182/blood-2008-01-133181
- Vesely S.K., George J.N., Lämmle B., Studt J.D., Alberio L., El-Harake M.A., Raskob G.E. ADAMTS13-activity in thrombotic thrombocytopenic purpura-haemolytic uremic syndrome relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003; 102(1): 60-8. DOI: 10.1182/blood-2003-01-0193
- Fuchigami S., Kaikita K., Soejima K., Matsukawa M., Honda T., Tsujita K. et al. Changes in plasma von Willebrand factor-cleaving protease(ADAMTS13) levels in patients with unstable angina. Thromb. Res. 2008; 122(5): 618-23. https://doi.org/10.1016/j.thromres.2007.12.025
- Fujikawa K., Suzuki H., McMullen B., Chung D. Purification of human von Willebrand factorcleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001; 98(6): 1662-6. DOI: 10.1182/blood.v98.6.1662
- Gerritsen H.E., Robles R., Lämmle B., Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood. 2001; 98(6): 1654-61. DOI: 10.1182/blood.v98.6.1654
- Levy G.G., Nichols W.C., Lian E.C., Foroud T., McClintick J.N., McGee B.M. et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001; 413(6855): 488-94. DOI: 10.1038/35097008
- Zheng X.L. Structure-function and regulation of ADAMTS-13 protease. J. Thromb. Haemost. 2013; 11(Suppl. 1): 11-23. doi: 10.1111/jth.12221.
- Tsai H.M. Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency. Hematol. Oncol. Clin. North Am. 2007; 21(4): 609-32. DOI: 10.1016/j.hoc.2007.06.003
- Müller-Deile J., Schiffer M. Renal involvement in preeclampsia: similarities to VEGF ablation therapy. J. Pregnancy. 2011; 2011: 176973. doi: 10.1155/2011/176973
- SoRelle R. Clopidogrel-associated thrombotic thrombocytopenic purpura identified. Circulation. 2000; 101(18): 9036-7. doi: 10.1592/phco.24.6.664.34732
- 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. DOI: 10.1056/NEJM199811263392203
- Furlan M., Robles R., Galbusera M., Remuzzi G., Kyrle P.A., 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. DOI: 10.1056/NEJM199811263392202
- Sadler J.E. What’s new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura. Am. Soc. Hematol. Educ. Program. 2015; 2015: 631-6. doi: 10.1182/asheducation-2015.1.631.
- George J.N., Nester C.M. Syndromes of thrombotic microangiopathy. N. Engl. J. Med. 2014; 371(7): 654-66. . doi: 10.1056/NEJMra1312353.
- Joly B.S., Coppo P., Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017; 129(21): 2836-46. doi: 10.1182/blood-2016-10-709857.
- Veyradier A., Lavergne J.M., Ribba A.S., Obert B., Loirat C., Meyer D. et al. Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome). J. Thromb. Haemost. 2004; 2(3): 424-9. DOI: 10.1111/j.1538-7933.2004.00623.x
- Moatti-Cohen M., Garrec C., Wolf M., Boisseau P., Galicier L., Azoulay E. et al. Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. Blood. 2012; 119(24): 5888-97. doi: 10.1182/blood-2012-02-408914
- Mariotte E., Azoulay E., Galicier L., Rondeau E., Zouiti F., Boisseau P. et al. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016; 3(5): e237-45. doi: 10.1016/S2352-3026(16)30018-7
- George J.N. Forecasting the future for patients with hereditary TTP. Blood. 2012; 120(2): 243-4. doi: 10.1182/blood-2012-05-427419
- Mansouri Taleghani M., von Krogh A.S., Fujimura Y., George J.N., Hrachovinová I., Knöbl P.N. et al. Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry. Hamostaseologie. 2013; 33(2): 138-43. doi: 10.5482/HAMO-13-04-0026.
- Joly B.S., Stepanian A., Leblanc T., Hajage D., Chambost H., Harambat J. et al. Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016; 3(11): e537-46. doi: 10.1186/s13256-018-1806-9
- Kwok S.K., Ju J.H., Cho C.S., Kim H.Y., Park S.H. Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus. 2009; 18(1): 16-21. doi: 10.1177/0961203308094360
- Ostendorf T., Van Roeyen C., Westenfeld R., Gawlik A., Kitahara M., De Heer E. et al. Inducible nitric oxide synthase-derived nitric oxide promotes glomerular angiogenesis via upregulation of vascular endothelial growth factor receptors. J. Am. Soc. Nephrol. 2004; 15(9): 2307-19. https://doi.org/10.1097/01.ASN.0000136425.75041.9C
- Yuan H.T., Li X.Z., Pitera J.E., Long D.A., Woolf A.S. Peritubular capillary loss after mouse acute nephrotoxicity correlates with down-regulation of vascular endothelial growth factor-A and hypoxia-inducible factor-1 alpha. Am. J. Pathol. 2003; 163(6): 2289-301. DOI: 10.1016/s0002-9440(10)63586-9
- Franchini M., Montagnana M., Targher G., Lippi G. Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other disease. Semin. Thromb. Hemost. 2007; 33(8): 787-97. DOI: 10.1055/s-2007-1000365
- Austin S.K., Starke R.D., Lawrie A.S., Cohen H., Machin S.J., Mackie I.J. The VWF/ ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction. Br. J. Haematol. 2008; 141(4): 536-44. doi: 10.1111/j.1365-2141.2008.07074.x.
- de Carvalho J.F., Freitas C.A., Lima I.V., Leite C.C., Lage L.V. Primary antiphospholipid syndrome with thrombotic thrombocytopenic purpura: a very unusual association. Lupus. 2009; 18(9): 841-4. . doi: 10.1177/0961203308101958.
- Grall M., Azoulay E., Galicier L., Provôt F., Wynckel A., Poullin P. et al. Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: Causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre. Am. J. Hematol. 2017; 92(4): 381-7. doi: 10.1002/ajh.24665.
- Kokame K., Nobe Y., Kokubo Y., Okayama A., Miyata T. FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay. Br. J. Haematol. 2005; 129(1): 93-100. DOI: 10.1111/j.1365-2141.2005.05420.x
- Obert B., Tout H., Veyradier A., Fressinaud E., Meyer D., Girma J.P. Estimation of the von Willebrand factor-cleaving protease in plasma using monoclonal antibodies to vWF. Thromb. Haemost. 1999; 82(5): 1382-5. PMID: 10595622
- Gerritsen H.E., Turecek P.L., Schwarz H.P., Lämmle B., Furlan M. Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP). Thromb. Haemost. 1999; 82(5): 1386-9. PMID: 10595623
- Andrew M., Paes B., Milner R., Johnston M., Mitchell L., Tollefsen D.M., Powers P. Development of the human coagulation system in the full-term infant. Blood. 1987; 70(1): 165-72. PMID: 3593964
- Andrew M., Vegh P., Johnston M., Bowker J., Ofosu F., Mitchell L. Maturation of the hemostatic system during childhood. Blood. 1992; 80(8): 1998-2005. PMID: 1391957
- Andrew M., Paes B., Milner R., Johnston M., Mitchell L., Tollefsen D.M. et al. Development of the human coagulation system in the healthy premature infant. Blood. 1988; 72(5): 1651-7. doi: 10.3389/fped.2017.00136
- Ehrenforth S., Junker R., Koch H.G., Kreuz W., Munchow N., Scharrer I., Nowak-Gottl U. Multicentre evaluation of combined prothrombotic defects associated with thrombophilia in childhood. Childhood Thrombophilia Study Group. Eur. J. Pediatr. 1999; 158(Suppl. 3): S97-104. DOI: 10.1007/pl00014359
- Thomas K.B., Sutor A.H., Altinkaya N., Grohmann A., Zehenter A., Leititis J.U. von Willebrand factor-collagen binding activity is increased in newborns and infants. Acta Paediatr. 1995; 84(6): 697-9. DOI: 10.1111/j.1651-2227.1995.tb13733.x
- Hellstrom-Westas L., Ley D., Berg A.C., Kristoffersson A.C., Holmberg L. VWF-cleaving protease (ADAMTS13) in premature infants. Acta Paediatr. 2005; 94(2): 205-10. doi: 10.1371/journal.pone.0118573
- Strauss T., Elisha N., Ravid B., Rosenberg N., Lubetsky A., Levy-Mendelovich S. et al. Activity of Von Willebrand factor and levels of VWF-cleaving protease (ADAMTS13) in preterm and full term neonates. Blood Cells Mol. Dis. 2017; 67: 14-7. doi: 10.1016/j.bcmd.2016.12.013
- Kulkarni A.A., Osmond M., Bapir M., Riddell A., Smith C., Lee C.A., Kadir R.A. The effect of labour on the coagulation system in the term neonate. Haemophilia. 2013; 19(4): 533-8.
- Feys H.B., Canciani M.T., Peyvandi F., Deckmyn H., Vanhoorelbeke K., Mannucci P.M. ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis. Br. J. Haematol. 2007; 138(4): 534-40. DOI: 10.1111/j.1365-2141.2007.06688.x
- Kavakli K., Canciani M.T., Mannucci P.M. Plasma levels of the von Willebrand factor-cleaving protease in physiological and pathological conditions in children. Pediatr. Hematol. Oncol. 2002; 19(7): 467-73. DOI: 10.1080/08880010290097288
- Mannucci P.M., Canciani M.T., Forza I., Lussana F., Lattuada A., Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood. 2001; 98(9): 2730-5. DOI: 10.1182/blood.v98.9.2730
- Schmugge M., Dunn M.S., Amankwah K.S., Blanchette V.S., Freedman J., Rand M.L. The activity of the von Willebrand factor cleaving protease ADAMTS-13 in newborn infants. J. Thromb. Haemost. 2004; 2(2): 228-33.
- Tsai H.M., Sarode R., Downes K.A. Ultralarge von Willebrand factor multimers and normal ADAMTS13 activity in the umbilical cord blood. Thromb. Res. 2002; 108(2-3): 121-5. DOI: 10.1016/s0049-3848(02)00396-1
- Reiter R.A., Varadi K., Turecek P.L., Jilma B., Knobl P. Changes in ADAMTS13 (vonWillebrand-factor-cleaving protease) activity after induced release of von Willebrand factor during acute systemic inflammation. Thromb. Haemost. 2005; 93(3): 554-8. DOI: 10.1160/TH04-08-0467
- Hunt R., Hoffman C.M., Emani S., Trenor C.C. 3rd, Emani S.M., Faraoni D. et al. Elevated preoperative von Willebrand factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease. J. Thromb. Haemost. 2017; 15(12): 2306-16. https://doi.org/10.1111/jth.13860
- Rock G.A., Shumak K.H., Buskard N.A., Blanchette V.S., Kelton J.G., Nair R.C. et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N. Engl. J. Med. 1991; 325(6): 393-7. DOI: 10.1056/NEJM199108083250604
- Schmidt B., Andrew M. Neonatal thrombosis: report of a prospective Canadian and international registry. Pediatrics. 1995; 96(5, Pt 1): 939-43. PMID: 7478839
- Chalmers E.A. Neonatal thrombosis. J. Clin. Pathol. 2000; 53(6): 419-23. DOI: 10.1136/jcp.53.6.419
- Chalmers E.A. Epidemiology of venous thromboembolism in neonates and children. Thromb. Res. 2006; 118(1): 3-12. . doi: 10.1177/1076029618814353
- Saracco P., Bagna R., Gentilomo C., Margarotto M., Viano A., Magnetti F. et al.; neonatal Working Group of the Registro Italiano Trombosi Infantili (RITI). Clinical data of neonatal systemic thrombosis. J. Pediatr. 2016; 171: 60-6. el. doi: 10.1016/j.jpeds.2015.12.035.
- Lotta L.A., Wu H.M., Mackie I.J., Noris M., Veyradier A., Scully M.A. et al. Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura. Blood. 2012; 120(2): 440-8. doi: 10.1182/blood-2012-01-403113
- Fujimura Y., Matsumoto M., Isonishi A., Yagi H., Kokame K., Soejima K. et al. Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan. J. Thromb. Haemost. 2011; 9(Suppl. 1): 283-301. doi: 10.1111/j.1538-7836.2011.04341.x.
- Schneppenheim R., Budde U., Oyen F., Angerhaus D., Aumann V., Drewke E. et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood. 2003; 101(5): 1845-50. DOI: 10.1182/blood-2002-08-2399
- Scully M., McDonald V., Cavenagh J., Hunt B.J., Longair I., Cohen H., et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011; 118(7): 1746-53. doi: 10.1182/blood-2011-03-341131.
Поступила 27.09.2019
Принята в печать 04.10.2019
Об авторах / Для корреспонденции
Бицадзе Виктория Омаровна, д.м.н., профессор РАН, профессор кафедры акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М. Сеченова. Тел.: +7(926)231-3829. Е-mail: gemostasis@mail.ru.Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Григорьева Кристина, студентка Института здоровья детей Первого МГМУ им. И.М. Сеченова. Е-mail: grigkristik96@gmail.com.
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Илалами Исмаил, PhD, MD, профессор университетской клиники Тенон, медицинский факультет университета Сорбонны, Франция, профессор кафедры акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М.Сеченова. Е-mail: ismail.elalamy@aphp.fr.
Макацария Александр Давидович, д.м.н., академик РАН, профессор, заведующий кафедрой акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М. Сеченова. Тел.: +7 (903)728-0897. Е-mail: gemostasis@mail.ru.
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Мингалимов Марат, студент Института здоровья детей Первого МГМУ им. И.М. Сеченова. Е-mail: ya.mingalimov2017@yandex.ru
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Воробьев Александр Викторович, к.м.н., доцент кафедры акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М. Сеченова Тел.: +7(903)105-6365; Е-mail: alvorobev@gmail.com.
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Хизроева Джамиля Хизриевна, д.м.н., профессор кафедры акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М. Сеченова.
Тел.: +7 (915)361-9073. Е-mail: gemostasis@mail.ru.
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Мэн Муян, аспирант кафедры акушерства и гинекологии Института здоровья детей Первого МГМУ им. И.М.Сеченова. Е-mail: gemostasis@mail.ru
Адрес: 109004, Москва, ул. Земляной Вал, д. 62, стр. 1.
Для цитирования: Бицадзе В.О., Григорьева К.Н., Илалами И., Макацария А.Д., Мингалимов М.А., Воробьев А.В., Хизроева Д.Х.,Мэн М. Клиническое значение определения металлопротеиназы ADAMTS-13, его ингибитора и фактора фон Виллебранда при патологических состояниях в неонатальном периоде.
Акушерство и гинекология. 2019; 11:74-81
https://dx.doi.org/10.18565/aig.2019.11.74-81