Thrombotic Microangiopathies

  • C. Adrie
  • E. Azoulay


Since the first description in 1925, thrombotic microangiopathies (TMA) have led to one of the most exciting fields of ongoing research to elucidate the pathophysiologic mechanisms responsible for this disorder. We shall review some of the classic clinical knowledge and more recent breakthroughs in the understanding of the mechanisms involved and the controversial treatments.


Thrombotic Thrombocytopenic Purpura Platelet Transfusion Simplify Acute Physiology Score Thrombotic Microangiopathy Plasma Infusion 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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  1. 1.
    Moschowitz E (1924) Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc 24: 21–24Google Scholar
  2. 2.
    Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R (1955) Hämolytisch-urämische Syndrome: Bilaterale Nierenrindennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr 85: 905–909PubMedGoogle Scholar
  3. 3.
    Remuzzi G (1987) HUS and TTP: variable expression of a single entity. Kidney Int 32: 292–308PubMedCrossRefGoogle Scholar
  4. 4.
    Ruggenenti P, Lutz J, Remuzzi G (1997) Pathogenesis and treatment of thrombotic microangiopathy. Kidney Int Suppl 58: 97–101Google Scholar
  5. 5.
    Drummond KN (1985) Hemolytic uremic syndrome-then and now. N Engl J Med 312: 116–118PubMedCrossRefGoogle Scholar
  6. 6.
    Moake JL (1998) von Willebrand factor in the pathophysiology of thrombotic thrombocytopenic purpura. Clin Lab Sci 11:362–364PubMedGoogle Scholar
  7. 7.
    Rock GA (2000) Management of thrombotic thrombocytopenic purpura. Br J Haematol 109: 496–507PubMedCrossRefGoogle Scholar
  8. 8.
    Moake JL (1997) Studies on the pathophysiology of thrombotic thrombocytopenic purpura. Semin Hematol 34: 83–89PubMedGoogle Scholar
  9. 9.
    Furlan M, Robles R, Galbusera M, et al (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339: 1578–1584Google Scholar
  10. 10.
    Tsai HM, Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339: 1585–1594PubMedCrossRefGoogle Scholar
  11. 11.
    Tandon NN, Rock G, Jamieson GA (1994) Anti-CD36 antibodies in thrombotic thrombocytopenic purpura. Br J Haematol 88: 816–825PubMedCrossRefGoogle Scholar
  12. 12.
    Leung DY, Moake JL, Havens PL, Kim M, Pober JS (1988) Lytic anti-endothelial cell antibodies in haemolytic-uraemic syndrome. Lancet 2: 183–186PubMedCrossRefGoogle Scholar
  13. 13.
    Dang CT, Magid MS, Weksler B, Chadburn A, Laurence J (1999) Enhanced endothelial cell apoptosis in splenic tissues of patients with thrombotic thrombocytopenic purpura. Blood 93: 1264–170PubMedGoogle Scholar
  14. 14.
    Remuzzi G, Ruggenenti P (1995) The hemolytic uremic syndrome. Kidney Int 48: 2–19PubMedCrossRefGoogle Scholar
  15. 15.
    Monnens L, Molenaar J, Lambert PH, Proesmans W, van Munster P (1980) The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 13: 168–171PubMedGoogle Scholar
  16. 16.
    Taylor CMT (2001) Hemolytic-uremic syndrome and complement factor H deficiency: clinical aspects. Semin Thromb Hemost 27: 185–190PubMedCrossRefGoogle Scholar
  17. 17.
    Le Gall JR, Lemeshow S, Saulnier F (1993) A new Simplified Acute Physiology Score ( SAPS II) based on a European/North American multicenter study. JAMA 270: 2957–2963PubMedCrossRefGoogle Scholar
  18. 18.
    Le Gall JR, Klar J, Lemeshow S, et al (1996) The Logistic Organ Dysfunction system. A new way to assess organ dysfunction in the intensive care unit. ICU Scoring Group. JAMA 276: 802–810PubMedCrossRefGoogle Scholar
  19. 19.
    Pene F, Papo T, Brudy-Gulphe L, et al (2001) Septic shock and thrombotic microangiopathy due to Mycobacterium tuberculosis in a nonimmunocompromised patient. Arch Intern Med 161: 1347–1348PubMedCrossRefGoogle Scholar
  20. 20.
    Byrnes JJ, Khurana M (1977) Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med 297: 1386–1389PubMedCrossRefGoogle Scholar
  21. 21.
    Bukowski RM, Hewlett JS, Harris JW, et al (1976) Exchange transfusions in the treatment of thrombotic thrombocytopenic purpura. Semin Hematol 13: 219–232PubMedGoogle Scholar
  22. 22.
    Shepard KV, Bukowski RM (1987) The treatment of thrombotic thrombocytopenic purpura with exchange transfusions, plasma infusions, and plasma exchange. Semin Hematol 24: 178–193PubMedGoogle Scholar
  23. 23.
    Rock GA, Shumak KH, Buskard NA, et al (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 325: 393–397PubMedCrossRefGoogle Scholar
  24. 24.
    Novitzky N, Jacobs P, Rosenstrauch W (1994) The treatment of thrombotic thrombocytopenic purpura: plasma infusion or exchange? Br J Haematol 87: 317–320PubMedCrossRefGoogle Scholar
  25. 25.
    Fakhouri F, Vincent F, Legendre C (2000) Pathological and therapeutic distinctions in HUS/TTP. Lancet 355: 497PubMedCrossRefGoogle Scholar
  26. 26.
    Ruggenenti P, Galbusera M, Cornejo RP, Bellavita P, Remuzzi G (1993) Thrombotic thrombocytopenic purpura: evidence that infusion rather than removal of plasma induces remission of the disease. Am J Kidney Dis 21: 314–318PubMedGoogle Scholar
  27. 27.
    Bell WR, Braine HG, Ness PM, Kickler TS (1991) Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 325: 398–403PubMedCrossRefGoogle Scholar
  28. 28.
    Dundas S, Murphy J, Soutar RL, et al (1999) Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet 354: 1327–1330PubMedCrossRefGoogle Scholar
  29. 29.
    Remuzzi G, Ruggenenti P (1998) The hemolytic uremic syndrome. Kidney Int Suppl 66: S54–S57PubMedGoogle Scholar
  30. 30.
    Moake JL (1994) Haemolytic-uraemic syndrome: basic science. Lancet 343: 393–397PubMedCrossRefGoogle Scholar
  31. 31.
    Boyce TG, Swerdlow DL, Griffin PM (1995) Escherichia coli O157:H7 and the hemolyticuremic syndrome. N Engl J Med 333: 364–368PubMedCrossRefGoogle Scholar
  32. 32.
    Jeejeebhoy FM, Zaltzman JS (1998) Thrombotic microangiopathy in association with cytomegalovirus infection in a renal transplant patient: a new treatment strategy. Transplantation 65: 1645–1648PubMedCrossRefGoogle Scholar
  33. 33.
    Satoh K, Takahashi H, Nagai K, Shibata A (1988) Thrombotic thrombocytopenic purpura and herpes zoster infection. Ann Intern Med 108: 154–155PubMedCrossRefGoogle Scholar
  34. 34.
    Hymes KB, Karpatkin S (1997) Human immunodeficiency virus infection and thrombotic microangiopathy. Semin Hematol 34: 117–125PubMedGoogle Scholar
  35. 35.
    Wasserstein A, Hill G, Goldfarb S, Goldberg M (1981) Recurrent thrombotic thrombocytopenic purpura after viral infection. Clinical and histologic simulation of chronic glomerulonephritis. Arch Intern Med 141: 685–687Google Scholar
  36. 36.
    Miniero R, Nesi F, Vai S, et al (1997) Cryptococcal meningitis following a thrombotic microangiopathy in an unrelated donor bone marrow transplant recipient. Pediatr Hematol Oncol 14: 469–474PubMedCrossRefGoogle Scholar
  37. 37.
    Creager AJ, Brecher ME, Bandarenko N (1998) Thrombotic thrombocytopenic purpura that is refractory to therapeutic plasma exchange in two patients with occult infection. Transfusion 38: 419–423PubMedCrossRefGoogle Scholar
  38. 38.
    Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI (2000) The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med 342: 1930–1936PubMedCrossRefGoogle Scholar
  39. 39.
    Cimolai N, Carter JE, Morrison BJ, Anderson JD (1990) Risk factors for the progression of Escherichia coli O157:H7 enteritis to hemolytic-uremic syndrome. J Pediatr 1990: 589–592Google Scholar
  40. 40.
    Cimolai N, Morrison S, Carter JE (1992) Risk factors for the central nervous system manifestations of gastroenteritis-associated hemolytic-uremic syndrome. Pediatrics 90: 616–621PubMedGoogle Scholar
  41. 41.
    Cimolai N, Basalyga S, Mah DG, Morrison BJ, Carter JE (1994) A continuing assessment of risk factors for the development of Escherichia coli O157:H7-associated hemolytic uremic syndrome. Clin Nephrol 42: 85–89PubMedGoogle Scholar
  42. 42.
    Harkness DR, Byrnes JJ, Lian EC, Williams WD, Hensley GT (1981) Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 246: 1931–1933PubMedCrossRefGoogle Scholar
  43. 43.
    Gordon LI, Kwann HC, Rossi EC (1987) Deleterious effects of platelet transfusion and recovery in patients with thrombotic microangiopathy. Semin Hematol 24: 194–201PubMedGoogle Scholar
  44. 44.
    Coppo P, Lassoued K, Mariette X, et al. (2001) Effectiveness of platelet transfusions after plasma exchange in adult thrombotic thrombocytopenic purpura: a report of two cases. Am J Hematol 68: 198–201PubMedCrossRefGoogle Scholar
  45. 45.
    Georges JN (2000) How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood 96: 1223–1229Google Scholar

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© Springer Science+Business Media New York 2002

Authors and Affiliations

  • C. Adrie
  • E. Azoulay

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