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Том 14 №2 2012 год - Нефрология и диализ

Тромботическая тромбоцитопеническая пурпура; случай успешного лечения


Иванова Е.С. Томилина Н.А. Подкорытова О.Л. Артюхина Л.Ю.

Аннотация: Тромботическая тромбоцитопеническая пурпура (ТТП) является редким, угрожающим жизни заболеванием, которое характеризуется микроангиопатической гемолитической анемией, тромбоцитопенией, неврологическими нарушениями, почечной недостаточностью и лихорадкой. В основе патогенеза ТТП – образование в артериолах и капиллярах тромбоцитарных тромбов вследствие тяжелого дефицита специфической металлопротеазы, ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type I motif 13), расщепляющей сверхкрупные мультимеры фактора Виллебранда в плазме крови, что необходимо для предупреждения спонтанного тромбообразования в системе микроциркуляции. Прогноз ТТП значительно улучшился после введения в клиническую практику инфузий свежезамороженной плазмы или плазмообмена, которые привели к снижению летальности при этом заболевании с 90% до менее 20%. В настоящем сообщении приводится клиническое наблюдение 39-летней женщины с тяжелым течением острой ТТП, успешно леченной сеансами плазмообмена. В статье также приведены последние данные о патогенезе и лечении ТТП.

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Ключевые слова: тромботическая тромбоцитопеническая пурпура, гемолитико-уремический синдром, тромботическая микроангиопатия, фактор Виллебранда, ADAMTS 13, плазмообмен, иммуносупрессивные препараты, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, thrombotic microangiopathy, von Willebrand factor, ADAMTS 13, plasma exchange, immunosuppressive agents

Список литературы:
  1. 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. Vol. 120. P. 556–573.
  2. Amorosi E.L., Ultmann J.E. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature // Medicine (Baltimore). 1966. P. 139–159.
  3. Andre P. P-selectin in haemostasis // Br J Haematol. 2004. Vol. 126. P. 298–306.
  4. Apte S.S. A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type I motifs: The ADAMTS family // Int J Biochem Cell Biol. 2004. Vol. 36. P. 981–985.
  5. Arya M., Anvari B., Romo G.M., et al. Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: Studies using optical tweezers // Blood. 2002. Vol. 99. P. 3971–3977.
  6. Bell W.R., Braine H.G., Ness P.M., et al. Improved survival in thrombotic thrombocytopenic purpura – hemolytic uremic syndrome. Clinical experience in 108 patients // N Engl J Med. 1991. Vol. 325. P. 398–403.
  7. Bernardo A., Ball C., Nolasco L., et al. Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow // Blood. 2004. Vol. 104. P. 100–106.
  8. Bohm M., Betz C., Miesbach W., et al. The course of ADAMTS 13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine // Br J Haematol. 2005. Vol. 129. P. 644–652.
  9. Bonthron D., Orr E.C., Mitsock L.M., et al. Nucleotide sequence of pre-pro-von Willebrand factor cDNA // Nucleic Acids Res. 1986. Vol. 14. P. 7125–7127.
  10. Bowen D.J.: An influence of ABO blood group on the rate of proteolysis of von Willebrand factor by ADAMTS 13 // J Thromb Haemost. 2003. Vol. 1. P. 33–40.
  11. Cataland S.R., Jin M., Ferketich A.K., et al. An evaluation of cyclosporine and corticosteroids individually as adjuncts to plasma exchange in the treatment of thrombotic thrombocytopenic purpura // Br J Haematol. 2007. Vol. 132 (1). P. 146–149.
  12. Cohen J.A., Brecher M.E., Bandarenko N. Cellular source of serum lactate dehydrogenase elevation in patients with thrombotic thrombocytopenic purpura // J Clin Apher. 1998. Vol. 13. P. 16–19.
  13. Cristophe O., Obert B., Meyer D., et al. The binding domain of von Willebrand factor to sulfatides is distinct from those interacting with glycoprotein Ib, heparin, and collagen and resides between amino acid residues Leu 512 and Lys 673 // Blood. 1991. Vol. 78. P. 2310–2317.
  14. Crowther M.A., Heddle N., Hayward C.P.M., et al. Splenectomy done during hematologic remission to prevent relapse in patients with thrombotic thrombocytopenic purpura // Ann Intern Med. 1996. Vol. 125. P. 294–296.
  15. De Groot P.G., Ottenhof-Rovers M., van Mouric J.A., et al. Evidence that the primary binding site of von Willebrand factor that mediates platelet adhesion on subendothelium is not collagen // J Clin Invest. 1988. Vol. 82. P. 65–73.
  16. Dong J.F., Moake J.L., Bernardo A., et al. ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor // J Biol Chem. 2003. Vol. 278. P. 29633–29639.
  17. Dong J.F., Moake J.L., Nolasco L., et al. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions // Blood. 2002. Vol. 100. P. 4033–4039.
  18. Fujikawa K., Suzuki H., McMullen B., et al. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family // Blood. 2001. Vol. 98. P. 1662–1666.
  19. Fujimura Y., Matsumoto M., Yagi H, et al. Von Willebrand factor-cleaving protease and Upschaw-Schulman syndrome // Int J Hematol. 2002. Vol. 75. P. 25–34.
  20. Fujimura Y., Titani K., Holland L.Z., et al. A heparin-binding domain of human von Willebrand factor. Characterization and localization to a tryptic fragment extending from amino acid residue. Val-449 to Lys-728 // J Biol Chem. 1987. Vol. 262. P. 1734–1739.
  21. Fujimura Y. Is Upshaw-Schulman syndrome congenital thrombotic thrombocytopenic purpura or hemolytic uremic syndrome? Yes to both // J Thromb Haemost. 2003. Vol. 1. P. 2457–2458.
  22. Furlan M., Robles R., Solenthaler M., et al. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura // Blood. 1997. Vol. 89. P. 3097–3103.
  23. Furlan M. Von Willebrand factor: Molecular size and functional activity // Ann Hematol. 1996. Vol. 72. P. 341–348.
  24. George J.N. How I treat patients with thrombotic thrombocytopenic purpura: 2010 // Blood. 2010. Vol. 116. P. 4060–4069.
  25. George J.N. The association of pregnancy with thrombotic thrombocytopenic purpura – haemolytic uremic syndrome // Curr Opin Hematol. 2003. Vol. 10. P. 339–344.
  26. George J.N. Thrombotic thrombocytopenic purpura // N Eng J Med. 2006. Vol. 354 (18). P. 1927–1935.
  27. George J.N.: The association of pregnancy with thrombotic thrombocytopenic purpura – hemolytic uremic syndrome // Curr Opin Hematol. 2003. Vol. 10. P. 339–344.
  28. Gerritsen H.E., Robles R., Lammle B., et al. Partial amino acid sequence of purified von Willebrand factor-cleaving protease // Blood. 2001. Vol. 98. P. 1654–1661.
  29. Houdijk W.P., Sakariassen K.S., Nievelstein P.F., et al. Role of factor VIII – von Willebrand factor and fibronectin in the interaction of platelets in flowing blood with monomeric and fibrillar human collagen types I and III // J Clin Invest. 1985. Vol. 75. P. 531–540.
  30. Konstantopoulos K., Chow T.W., Turner N.A., et al. Shear stress-induced binding of von Willebrand factor to platelets // Biorheology. 1997. Vol. 34. P. 57–71.
  31. Kremer Hovinga J.A., Vesely S.K., Terrell D.R., et al. Survival and relapse in patients with thrombotic thrombocytopenic purpura // Blood. 2010. Vol. 115 (8). P. 1500–1511.
  32. Levy G.G., Nichols W.C., Lian E.C., et al. Mutation in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura // Nature. 2001. Vol. 413. P. 488–494.
  33. Ling H.T., Field J.J., Blinder M.A. Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura: a report of 13 cases and review of the literature // Am J Hematol. 2009. Vol. 84 (7). P. 418–421.
  34. Mancuso D.J., Tuley E.A., Westfield L.A., et al. Structure of the gene for human von Willebrand factor // J Biol Chem. 1989. Vol. 264. P. 19514–19527.
  35. Mayadas T.N., Wagner D.D. In vitro multimerization of von Willebrand factor is triggered by low pH. Importance of the propolypeptide and free sulfhydryls // J Biol Chem. 1989. Vol. 264. P. 13497–13503.
  36. Medina P.J., Sipols J.M., George J.N. Drug-associated thrombotic thrombocytopenic purpura – hemolytic uremic syndrome // Curr Opin Hematol. 2001. Vol. 8. P. 286–293.
  37. Moake J.L. Thrombotic microangiopathies // N Eng J Med. 2002. Vol. 347. P. 589–600.
  38. Moake J.L. Thrombotic thrombocytopenic purpura: the systemic clumping “plague” // Annu Rev Med. 2002. Vol. 53. P. 75–88.
  39. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries, an undescribed disease // Arch Intern Med. 1925. Vol. 36. P. 89–93.
  40. Mount D., Pollak M. Molecular and Genetic Basis of Renal Disease. / Matouk C.C., Marsden P.A. Molecular Insights into the Thrombotic Microangiopathies // Saunders, 2007. P. 453–480.
  41. Nguyen L., Terrell D.R., Duvall D., et al. Complications of plasma exchange in patients treated for thrombotic thrombocytopenic purpura: IV. An additional study of 43 consecutive patients, 2005–2008 // Transfusion. 2009. Vol. 49 (2). P. 392–394.
  42. Nogami K., Shima M., Nishiya K., et al. A novel mechanism of factor VIII protection by von Willebrand factor from activated protein C-catalyzed inactivation // Blood. 2002. Vol. 99. P. 3993–3998.
  43. Noris M., Remuzzi G. Genetics and genetic testing in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura // Seminars in nephrology. 2010. Vol. 30 (4). P. 395–408.
  44. Padilla A., Moake J.L., Bernardo A., et al. P-selectin anchors newly released ultralarge von Willebrand factor multimers to the endothelial cell surface // Blood. 2004. Vol. 103. P. 2150–2156.
  45. Pimanda J., Hogg P. Control of von Willebrand factor multimer size and implications for disease // Blood Rev. 2002. Vol. 16. P. 185–192.
  46. Pimanda J.E., Ganderton T., Maekawa A., et al. Role of thrombospondin-1 in control of von Willebrand factor multimer size in mice // J Biol Chem. 2004. Vol. 279. P. 21439–21448.
  47. Raife T.J., Friedman K.D., Dwyre D.M. The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma // Transfusion. 2006. Vol. 46. P. 74–79.
  48. Richardson M.W., Allen G.A., Monahan P.E. Thrombosis in children: current perspective and distinct challenges // Thromb Haemost. 2002. Vol. 88. P. 900–911.
  49. Rock G., Shumak K.H., Sutton D.M.C., et al. Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura // Br J Haematol. 1996. Vol. 94. P. 383–386.
  50. Rock G.A., Shumak K.N., Buskard N.A., et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group // N Eng J Med. 1991. Vol. 325. P. 393–397.
  51. Ruggery Z.M. Von Willebrand factor, platelets and endothelial cell interactions // J Thromb Haemost. 2003. Vol. 1. P. 1335–1342.
  52. Ruggery Z.M. Von Willebrand factor // J Clin Invest. 1997. Vol. 99. P. 559–564.
  53. Sadler J.E., Moake J.L., Miyata T., et al. Recent advances in thrombotic thrombocytopenic purpura. / In: Broudy V.C., Berliner N., Larson R.A., Leung L.L.K. // Hematology. 2004. Washington, D.C.: American Society of Hematology. 2004. P. 407–423.
  54. Savage B., Almus-Jacobs F., Ruggeri Z.M. Specific synergy of multiple substrate-receptor interaction in platelet thrombus formation under flow // Cell. 1998. Vol. 94. P. 657–666.
  55. Savage B., Sixma J.J., Ruggeri Z.M. Functional self-association of von Willebrand factor during platelet adhesion under flow // Proc Natl Acad Sci U S A. 2002. Vol. 99. P. 425–430.
  56. Scully M.F., Cohen H., Cavenagh J.D., et al. Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS 13 // Br J Haematol. 2006. Vol. 136 (3). P. 451–461.
  57. Siedlecki C.A., Lestini B.J., Kottke-Marchant K.K., et al. Shear-dependent changes in the three-dimensional structure of human von Willebrand factor // Blood. 1996. Vol. 88. P. 2939–2950.
  58. Smith J.W., Weinstein R. Therapeutic apheresis: a summary of current indication categories endorsed by the AABB and American Society for Apheresis // Transfusion. 2003. Vol. 43. P. 820–822.
  59. Souto J.C., Almasy L., Muniz-Diaz E., et al. Functional effects of the ABO locus polymorphism on plasma levels of von Willebrand factor, factor VIII, and activated partial thromboplastin time // Arterioscler Thromb Vasc Biol. 2000. Vol. 20. P. 2024–2028.
  60. Souto J.C., Almasy L., Soria J.M., et al. Genome-wide linkage analysis of von Willebrand factor plasma levels: Results from the GAIT project // Thromb Haemost. 2003. Vol. 89. P. 468–474.
  61. Sutor G.C., Schmidt R.E., Albrecht H. Thrombotic microangiopaties and HIV infection: Report of two typical cases, features of HUS and TTP, and review of the literature // Infection. 1999. Vol. 27. P. 12–15.
  62. Suzuki M., Murata M., Matsubara Y., et al. Detection of von Willebrand factor-cleaving protease (ADAMTS-13) in human platelets // Biochem Biophys Res Commun. 2004. Vol. 313. P. 212–216.
  63. Tangelder G.J., Slaaf D.W., Arts T., et al. Wall shear rate in arterioles in vivo: Least estimates from platelet velocity profiles // Am J Physiol. 1988. Vol. 254. P. H1059–H1064.
  64. Vesely S.K., Li X., McMinn J.R., et al. Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura – haemolytic uremic syndrome // Transfusion. 2004. Vol. 44. P. 1149–1158.
  65. Veyradier A., Lavergne J.M., Ribba A.S., et al. Ten candidate ADAMTS 13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upschaw–Schulman syndrome) // J Thromb Haemost. 2004. Vol. 2. P. 424–429.
  66. Wada H., Kaneko T., Ohiwa M., et al. Plasma cytokine levels in thrombotic thrombocytopenic purpura // Am J Hematol. 1992. Vol. 40. P. 167–170.
  67. Wagner D.D., Lawrence S.O., Ohlsson-Wilhelm B.M., et al. Topology and order of formation of interchain disulfide bonds in von Willebrand factor // Blood. 1987. Vol. 69. P. 27–32.
  68. Zeigler Z.R., Shadduck R.K., Gryn J.F., et al. Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP) // J Clin Apher. 2001. Vol. 16. P. 19–22.
  69. Zheng X., Chung D., Takayama T.K., et al. Structure of von Willebrand factor-cleaving protease (ADAMTS 13), a metalloprotease involved in thrombotic thrombocytopenic purpura // J Biol Chem. 2001. Vol. 276. P. 41059–41063.
  70. Zheng X., Majerus E.M., Sadler J.E. ADAMTS 13 and TTP // Curr Opin Hematol. 2002. Vol. 9. P. 389–394.
  71. Ziman A., Mitri M., Klapper E., et al. 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. Vol. 45. P. 41–49.

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