Thrombotic Microangiopathy Syndrome in the ICU

  • S. Samy Modeliar
  • M. Monge
  • M. Slama
Conference paper

Abstract

Since the first descriptions of haemolytic-uremic syndrome (HUS) by Moschowitz and thrombotic thrombocytopenic purpura (TTP) by Gasser, our knowledge about thrombotic microangiopathy (TMA) has grown considerably [1]. TMA now refers to a group of diseases comprising mechanical hemolytic anemia, peripheral thrombocytopenia, and varying degrees of organ failure. The incidence of TMA is increasing in the USA. Considerable progress has recently been made in the understanding of the pathophysiological mechanisms of TMA. These rare diseases, characterized by platelet thrombi in the microcirculation, are responsible for often serious organ dysfunction leading to the admission of these patients to intensive care units (ICUs). The prognosis of TMA was extremely poor prior to plasma therapy and especially plasma exchange. TMA is a serious, life-threatening disease that requires early diagnosis and urgent specialized therapeutic management.

Keywords

Human Immunodeficiency Virus Plasma Exchange Disseminate Intravascular Coagulation Hemolytic Uremic Syndrome Fresh Freeze Plasma 
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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References

  1. 1.
    Coppo P, Adrie C, Azoulay E, et al (2003) Infectious diseases as a trigger in thrombotic microangiopathies in intensive care unit (ICU) patients? Intensive Care Med 29:564–569PubMedGoogle Scholar
  2. 2.
    Coppo P, Vernant J, Veyradier A, et al (2005) Thrombotic thrombocytopenic purpura and other thrombotic microangiopathy syndromes. EMC-Hematologie 2:14–34CrossRefGoogle Scholar
  3. 3.
    Fujikawa K, Suzuki H, McMullen B, Chung D (2001) Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 98:1662–1666PubMedCrossRefGoogle Scholar
  4. 4.
    Gerritsen HE, Robles R, Lammle B, Furlan M (2001) Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 98:1654–1661PubMedCrossRefGoogle Scholar
  5. 5.
    Moake JL (2002) Thrombotic microangiopathies. N Engl J Med 347:589–600PubMedCrossRefGoogle Scholar
  6. 6.
    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 339:1578–1584PubMedCrossRefGoogle Scholar
  7. 7.
    Levy GG, Nichols WC, Lian EC, et al (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 413:488–494PubMedCrossRefGoogle Scholar
  8. 8.
    Remuzzi G, Galbusera M, Noris M, et al (2002) von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood 100:778–785PubMedCrossRefGoogle Scholar
  9. 9.
    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
  10. 10.
    Veyradier A, Obert B, Houllier A, et al (2001) Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood 98:1765–1772PubMedCrossRefGoogle Scholar
  11. 11.
    Pene F, Vigneau C, Auburtin M, et al (2005) Outcome of severe adult thrombotic microangiopathies in the intensive care unit. Intensive Care Med 31:71–78PubMedCrossRefGoogle Scholar
  12. 12.
    Hymes KB, Karpatkin S (1997) Human immunodeficiency virus infection and thrombotic microangiopathy. Semin Hematol 34:117–125PubMedGoogle Scholar
  13. 13.
    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
  14. 14.
    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
  15. 15.
    Moorthy B, Makker SP (1979) Hemolytic-uremic syndrome associated with pneumococcal sepsis. J Pediatr 95:558–559PubMedCrossRefGoogle Scholar
  16. 16.
    Riggs SA, Wray NP, Waddell CC, Rossen RD, Gyorkey F (1982) Thrombotic thrombocytopenic purpura complicating Legionnaires’ disease. Arch Intern Med 142:2275–2280PubMedCrossRefGoogle Scholar
  17. 17.
    Satoh K, Takahashi H, Nagai K, Shibata A (1988) Thrombotic thrombocytopenic purpura and herpes zoster infection. Ann Intern Med 108:154–155PubMedGoogle Scholar
  18. 18.
    Schroder S, Spyridopoulos I, Konig J, Jaschonek KG, Luft D, Seif FJ (1995) Thrombotic thrombocytopenic purpura (TTP) associated with a Borrelia burgdorferi infection. Am J Hematol 50:72–73PubMedCrossRefGoogle Scholar
  19. 19.
    Shalev O, Kami A, Kornberg A, Brezis M (1981) Thrombotic thrombocytopenic purpura associated with Bacteroides bacteremia. Arch Intern Med 141:692PubMedCrossRefGoogle Scholar
  20. 20.
    Toscano V, Bontadini A, Falsone G, et al (1995) Thrombotic thrombocytopenic purpura associated with primary tuberculosis. Infection 23:58–59PubMedCrossRefGoogle Scholar
  21. 21.
    Noris M, Remuzzi G (2005) Hemolytic uremic syndrome. J Am Soc Nephrol 16:1035–1050PubMedCrossRefGoogle Scholar
  22. 22.
    Rossert J, Fischer E (2001) Insuffisances rénales aiguës d’origine glomérulaire et vasculaire. In: Offenstadt G, Vachon F, Cardinaud JP, et al (eds) Réanimation Médicale. Masson, Paris, pp 1239–1248Google Scholar
  23. 23.
    Roodhooft AM, McLean RH, Elst E, et al (1990) Recurrent haemolytic uraemic syndrome and acquired hypomorphic variant of the third component of complement. Pediatr Nephrol 4:597–599PubMedCrossRefGoogle Scholar
  24. 24.
    Dragon-Durey MA, Fremeaux-Bacchi V, Loirat C, et al (2004) Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol 15:787–795PubMedCrossRefGoogle Scholar
  25. 25.
    Dragon-Durey MA, Loirat C, Cloarec S, et al (2005) Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 16:555–563PubMedCrossRefGoogle Scholar
  26. 26.
    Knobl P, Rintelen C, Kornek G, et al (1997) Plasma exchange for treatment of thrombotic hrombocytopenic purpura in critically ill patients. Intensive Care Med 23:44–50PubMedCrossRefGoogle Scholar
  27. 27.
    Tardy B (2001) Purpura thrombotique thrombocytopénique. In: Offenstadt G, Vachon F, Cardinaud JP, et al (eds) Réanimation Médicale. Masson, Paris, pp 1316–1319Google Scholar
  28. 28.
    Remuzzi G, Ruggenenti P (1995) The hemolytic uremic syndrome. Kidney Int 48:2–19PubMedCrossRefGoogle Scholar
  29. 29.
    Sadler JE, Moake JL, Miyata T, George JN (2004) Recent advances in thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program):407–423Google Scholar
  30. 30.
    Chandler WL, Jelacic S, Boster DR, et al (2002) Prothrombotic coagulation abnormalities preceding the hemolytic-uremic syndrome. N Engl J Med 346:23–32PubMedCrossRefGoogle Scholar
  31. 31.
    Bell WR, Braine HG, Ness PM, et al (1991) Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 325:398–403PubMedCrossRefGoogle Scholar
  32. 32.
    Lara PN, Jr, Coe TL, Zhou H, et al (1999) Improved survival with plasma exchange in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Am J Med 107:573–579PubMedCrossRefGoogle Scholar
  33. 33.
    Rock G, Shumak K, Kelton J, et al (1992) Thrombotic thrombocytopenic purpura: outcome in 24 patients with renal impairment treated with plasma exchange. Canadian Apheresis Study Group. Transfusion 32:710–714PubMedCrossRefGoogle Scholar
  34. 34.
    Pereira A, Mazzara R, Monteagudo J, et al (1995) Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange. Ann Hematol 70:319–323PubMedCrossRefGoogle Scholar
  35. 35.
    Rose M, Eldor A (1987) High incidence of relapses in thrombotic thrombocytopenic purpura. Clinical study of 38 patients. Am J Med 83:437–444PubMedCrossRefGoogle Scholar
  36. 36.
    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
  37. 37.
    Wong CS, Jelacic S, Habeeb RL, et al (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
  38. 38.
    Sarode R, Gottschall JL, Aster RH, et al (1997) Thrombotic thrombocytopenic purpura: early and late responders. Am J Hematol 54:102–107PubMedCrossRefGoogle Scholar
  39. 39.
    Yomtovian R, Niklinski W, Silver B, Sarode R, Tsai HM (2004) Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature. Br J Haematol 124:787–795PubMedCrossRefGoogle Scholar
  40. 40.
    Gaddis TG, Guthrie TH, Jr, Drew MJ, et al (1997) Treatment of plasma refractory thrombotic thrombocytopenic purpura with protein A immunoabsorption. Am J Hematol 55:55–58PubMedCrossRefGoogle Scholar
  41. 41.
    Bennett CL, Connors JM, Carwile JM, et al (2000) Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med 342:1773–1777PubMedCrossRefGoogle Scholar
  42. 42.
    Bennett CL, Weinberg PD, Rozenberg-Ben-Dror K, et al (1998) Thrombotic thrombocytopenic purpura associated with ticlopidine. A review of 60 cases. Ann Intern Med 128:541–544PubMedGoogle Scholar
  43. 43.
    Nagaya S, Wada H, Oka K, et al (1995) Hemostatic abnormalities and increased vascular endothelial cell markers in patients with red cell fragmentation syndrome induced by mitomycin C. Am J Hematol 50:237–243PubMedCrossRefGoogle Scholar
  44. 44.
    Maslo C, Peraldi MN, Desenclos JC, et al (1997) Thrombotic microangiopathy and cytomegalovirus disease in patients infected with human immunodeficiency virus. Clin Infect Dis 24:350–355PubMedGoogle Scholar

Copyright information

© Springer Science + Business Media Inc. 2006

Authors and Affiliations

  • S. Samy Modeliar
    • 1
  • M. Monge
    • 2
  • M. Slama
    • 3
  1. 1.Nephrology Department, Intensive Care UnitCHU Amiens — Hôpital SudAmiensFrance
  2. 2.Nephrology Internal Medicine DepartmentCHU Amiens — Hôpital SudAmiensFrance
  3. 3.Nephrology Internal Medicine Department, Intensive Care UnitCHU Amiens — Hôpital SudAmiensFrance

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