Similarities and Differences between the Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

  • J. J. Byrnes
Part of the Developments in Nephrology book series (DINE, volume 7)


Thrombotic thrombocytopenic purpura (TTP) and the hemolytic uremic syndrome (HUS) are both defined by a constellation of clinical manifestations. Precise definition of the essence of either TTP or HUS has not yet been realized. The clinical elements that constitute the HUS are contained in those that compose TTP. Due to this overlap in clinical manifestations, the distinction between the HUS and TTP may be difficult, especially in the adult presentations. Furthermore, platelet aggregation causing microvascular occlusion is an essential component of both disorders. The pathologic tissue abnormalities are similar; only the organ distribution is different. Some would argue that TTP and the HUS are simply variations of a common process. Others emphasize the heterogeneous aspects of both disorders. Until a better understanding of the predisposing factors, the precipitating mechanisms and the pathogenetic sequence is achieved, this argument cannot be resolved. The purpose here is to review the available information pertaining to these aspects of TTP and the HUS, to compare and contrast these data, and hopefully to provide a foundation of current knowledge from which to proceed in further analysis and understanding of these two important syndromes.


Platelet Aggregation Hemolytic Uremic Syndrome Thrombotic Thrombocytopenic Purpura Exchange Transfusion Haemolytic Uraemic Syndrome 
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.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Moschcowitz, E.: An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. Arch. Intern. Med. 36: 89, 1925.Google Scholar
  2. 2.
    Hosebye, K.O., Stickney, J.M. and Bennett, W.A.: Platelet thrombosis in leukemia. Ann. Intern. Med. 44: 975, 1956.Google Scholar
  3. 3.
    Ritama, V.: Granular capillary thrombi in acute familial leukemia. Ann. Med. Int. Fenn. 43: 305, 1954.PubMedGoogle Scholar
  4. 4.
    Komori, R.: Thrombotic microangiopathy in patients with malignant tumor. Acta Path. Jap. 12: 3: 379, 1962.Google Scholar
  5. 5.
    Jones, D.B. and Loring, W.E.: Glomerular thrombosis. Amer. J. Path. 27: 841, 1951.PubMedGoogle Scholar
  6. 6.
    Pagel, W.: Acronecrosis due to fibrin thrombi and endothelial cell thrombi. Amer. J. Med. Sci. 218: 425, 1949.PubMedCrossRefGoogle Scholar
  7. 7.
    Singer, K.: Thrombotic thrombocytopenic purpura. Adv. Intern. Med. 6: 195, 1954.PubMedGoogle Scholar
  8. Amorosi, E.L. and Ultman, J.E.: Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature. Medicine (Baltimore) 139, 1966.Google Scholar
  9. 9.
    Gasser, C., Gautier, E., Siebenmann, R.E. and Dechslin, R.: Hamolytisch-uramische Syndrome: bilaterale Nieren- rindennekrosen bei akuten erworbenen hamolytischen Anamen. Schweiz. Med. Wschr. 85: 905, 1955.PubMedGoogle Scholar
  10. 10.
    Gianantonio, C.A., Vitacco, M., Mendilaharzu, F. et al: The hemolytic uremic syndrome: renal status of 76 patients at long-term followup. J. Pediat. 72: 757, 1968.PubMedCrossRefGoogle Scholar
  11. 11.
    Goldstein, M.H., Churg, J., Strauss, L. and Gribetz, D.: Hemolytic-uremic syndrome. Nephron 23: 263, 1979.PubMedCrossRefGoogle Scholar
  12. 12.
    Kaplan, B.S., Thomson, P.D. and de Chaclarevian, J.P.: The hemolytic uremic syndrome. Pediatr. Clin. North. Am. 23: 761, 1976.PubMedGoogle Scholar
  13. 13.
    Dolislager, D. and Tune B.: The hemolytic-uremic syndrome- spectrum of severity and significance of prodrome. Am. J. Dis. Child. 132: 55, 1978.PubMedGoogle Scholar
  14. 14.
    O’Regan, S., Robitaille P., Mongeau, J.G. et al: The hemolytic uremic syndrome associated with ECHO 22 infection. Clin. Pediatr. 19: 125, 1980.Google Scholar
  15. 15.
    Raghupathy, P., Date, A., Shastry, J.C.M. et al: Haemo- lytic-uraemic syndrome complicating shigella dysentery in south Indian children. Br. Med. J. Is 1518f 1978.Google Scholar
  16. 16.
    Kaplan, B.S. and Drummond, K.N.: The hemolytic-uremic syndrome is a syndrome. N. Engl. J. Med. 298: 964, 1978.PubMedCrossRefGoogle Scholar
  17. 17.
    Harker, L.A. and Slichter, S.J.: Platelet and fibrinogen consumption in man. N. Engl. J. Med. 287: 999, 1972.PubMedCrossRefGoogle Scholar
  18. 18.
    Jaffe, E.A., Nachman, R.L. and Merskey, C.: Thrombocytopenic purpura. A coagulation parameter in twelve patients. Blood 42: 499, 1973.PubMedGoogle Scholar
  19. 19.
    Kwaan, H.C., Gallo, C., Potter, E. et al: The nature of the vascular lesion in thrombotic thrombocytopenic purpura. Ann. Intern. Med. 68: 1169, 1968.Google Scholar
  20. 20.
    Kwaan, H.C.: The pathogenesis of thrombotic thrombocytopenic purpura. Semin. Thromb. Hemostas. 5: 184, 1979.Google Scholar
  21. 21.
    Ekert, H., Friedlander, I. and Hardisty, R.M.: The role of platelets in fibrinolysis. Studies on the plasminogen activator and antiplasmin activity of platelets. Br. J. Haematol. 18: 575, 1970.PubMedCrossRefGoogle Scholar
  22. 22.
    Remuzzi, G., Misiani, R., Mecca, G. et al: Thrombocyto¬penic purpura - a deficiency of plasma factors regulating platelet action. N. Engl. J. Med. 299: 311, 1978.PubMedGoogle Scholar
  23. 23.
    Remuzzi G., Marchesi, D., Mecca, G. et al: Haemolytic- uraemic syndrome: deficiency of plasma factor(s) regulating prostacyclin activity. Lancet 2: 871, 1978.PubMedCrossRefGoogle Scholar
  24. 24.
    Mant, M.J., Cauchi, M.N. and Medley, G.: Thrombotic thrombocytopenic purpura: a report of a case with possible immune etiology. Blood 40: 416, 1972.PubMedGoogle Scholar
  25. 25.
    Burns ER, Zucker-Franklin D.: Pathologic effects of plasma from patients with thrombotic thrombocytopenic purpura on platelets and cultured vascular endothelial cells. Blood 60: 1030, 1982.PubMedGoogle Scholar
  26. 26.
    Neame, P.B., Hirsh, J., Browman, G. et al: Thrombotic thrombocytopenic purpura: a syndrome of intravascular platelet consumption. Can. Med. Assoc. J. 114: 1108, 1976.PubMedGoogle Scholar
  27. 27.
    Harkness, D.R., Byrnes, J.J., Lian, E.C-Y. et al: Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 246: 1931, 1981.PubMedCrossRefGoogle Scholar
  28. 28.
    Lian, E.C-Y., Harkness, D.R., Byrnes, J.J. et al: The presence of a platelet aggregating factor in the plasma of patients with thrombotic thrombocytopenic purpura. Clin. Res. 27: 780, 1979.Google Scholar
  29. 29.
    Lian, E.C-Y.: Effects of platelet inhibitors on platelet aggregation induced by plasma of patients with thrombotic thrombocytopenic purpura. Clin. Res. 27: 780, 1979.Google Scholar
  30. 30.
    Moake, J.L., Rudy, C.K., Troll, J.H. et al: Unusually large plasma factor. VIII. von Willebrand factor multi- mers in chronic relapsing thrombotic thrombocytopenic purpura. N. Engl. J. Med. 307: 1432, 1982.PubMedCrossRefGoogle Scholar
  31. 31.
    Pareti, F.I., Capitanio, A., Mannucci, L., Ponticelli, C. and Mannucci, P.M.: Acquired dysfunction due to the circulation of “exhausted” platelets. Am. J. Med. 69: 235, 1980.PubMedCrossRefGoogle Scholar
  32. 32.
    Kaplan, B.S. and Fong, J.S.C.: Reduced platelet aggre¬gation in hemolytic uremic syndrome. Thromb. Haemost. 43: 154, 1980.Google Scholar
  33. 33.
    Mettler, N.E.: Isolation of a microtatobiote from patients with hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and from mites in the United States. N. Engl. J. Med. 281: 1023, 1969.PubMedCrossRefGoogle Scholar
  34. 34.
    Berberich, F.R., Cuene, S.A., Chard, R.L. et al: Thrombotic thrombocytopenic purpura. Three cases with platelet and fibrinogen survival studies. J. Pediatr. 84: 503, 1974.PubMedCrossRefGoogle Scholar
  35. 35.
    Myers, T.J., Wakem, C.J., Ball, E.D. et al: Thrombotic thrombocytopenic purpura: Combined treatment with plasmapheresis and antiplatelet agents. Ann. Intern. Med. 92: (pt. 1) 149, 1980.PubMedGoogle Scholar
  36. 36.
    Reynolds, P.M., Jackson, J.M., Brine, J.A.S. et al: Thrombotic thrombocytopenic purpura - remission following splenectomy: report of a case and review of the literature. Am. J. Med. 61: 439, 1976.PubMedCrossRefGoogle Scholar
  37. 37.
    Brown, R.C., Blecher, T.E., French, E.A. et al: Thrombotic thrombocytopenic purpura after influenza vaccination. Br. Med. J. 2: 303, 1973.PubMedCrossRefGoogle Scholar
  38. 38.
    Frick, P.G. and Hitzig, W.H.: Simultaneous thrombotic thrombocytopenic purpura and agammaglobulinaemia. Lancet 2: 1401, 1960.CrossRefGoogle Scholar
  39. 39.
    Glasgow, L.A. and Balduzzi, P.: Isolation of Coxsackie virus group A type 4 from a patient with hemolytic uremic syndrome. N. Engl. J. Med. 273: 754, 1965.PubMedCrossRefGoogle Scholar
  40. 40.
    Austin, T.W. and Ray, C.G.: Coxsackie virus group B infections and the hemolytic-uremic syndrome. J. Infect. Dis. 127: 698, 1973.PubMedCrossRefGoogle Scholar
  41. 41.
    Koster, F., Levin, J., Walker, L. et al: Hemolytic- uremic syndrome after shigellosis: relation to endotox- emia and circulating immune complexes. N. Engl. J. Med. 298: 927, 1978.PubMedCrossRefGoogle Scholar
  42. Karmali, M.A., Steele, B.T., Petric, M. and Lim, C.: Sporadic cases of haemolytic-uraemic syndrome associated with faecal cytotoxin and cytotoxin-producing escherichia coli in stools. Lancet Is 619, 1983.Google Scholar
  43. 43.
    Boswell, D.C. and Sierra, G.: Probable 16-year course in a patient with thrombotic thrombocytopenic purpura. Illinois Med. J. 145: 29, 1974.Google Scholar
  44. 44.
    Upshaw, J.D., Jr.: Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N. Engl. J. Med. 298: 1350, 1978.PubMedCrossRefGoogle Scholar
  45. 45.
    May, H.V., Harbert, G.M. and Thornton, W.N.: Thrombotic thrombocytopenic purpura associated with pregnancy. Am. J. Obst. Gynecol. 126: 452, 1976.Google Scholar
  46. 46.
    Wunzel, J.W.: TTP lesions in placenta but not in fetus. N. Engl. J. Med. 301: 503, 1979.Google Scholar
  47. 47.
    Fuchs, W.E., George, J.N., Dotin, L.N. et al: Thrombotic thrombocytopenic purpura occurrence two years apart during late pregnancy in two sisters. JAMA 235: 2126, 1976.PubMedCrossRefGoogle Scholar
  48. 48.
    Piver, M.S. et al: Thrombotic thrombocytopenic purpura during pregnancy. Am. J. Obst. Gynecol. 100: 302, 1968.Google Scholar
  49. 49.
    Caggiano, V., Chosney, B. and Way, L.W.: Thrombotic thrombocytopenic purpura, cholangiocarcinoma, and oral contraceptives. Lancet 2: 365, 1980.PubMedCrossRefGoogle Scholar
  50. 50.
    Vesconi, S., Langer, M., Rossi, E. et al: Thrombotic thrombocytopenic purpura during oral contraceptive treatment. Thromb. Haemostas. (Stuttg.) 40: 563, 1979.Google Scholar
  51. 51.
    Robson, J.S., Martin, A.M., Ruckley, V.A. et al: Irreversible postpartum renal failure. Q. J. Med. 37: 423, 1968.Google Scholar
  52. Brown, C.B., Clarkson, A.R., Robson, J.S. et al: Hemolytic uremic syndrome in women taking oral contraceptives. Lancet Is 1479, 1973.Google Scholar
  53. 53.
    Wallace, D.C., Lovric, A., Clubb, J.S. et als Thrombotic thrombocytopenic purpura in four siblings. Am. J. Med. 38s 724, 1975.CrossRefGoogle Scholar
  54. 54.
    Paz, P.A., Elijovich, F. and Barcat, J.A.s Fatal simultaneous thrombocytopenic purpura in siblings. Br. Med. J. 4: 727, 1969.CrossRefGoogle Scholar
  55. 55.
    Watson, C.G. and Cooper, W.M.: Thrombotic thrombocytopenic purpura. Concomitant occurrence in husband and wife. JAMA 215: 1821, 1971.PubMedCrossRefGoogle Scholar
  56. 56.
    Hogewind, B.L., de la Riviere, G.B., van Eo, L.A. et al: Familial occurrence of the haemolytic uraemic syndrome. Acta Med. Scand. 207: 73, 1980.Google Scholar
  57. 57.
    Tune, B.M., Groshong, T., Plumer, L. et al: The hemo- lytic-uremic syndrome in siblings: A prospective survey. J. Pediatr. 85: 682, 1974.PubMedCrossRefGoogle Scholar
  58. 58.
    Hagge, W.W., Holley, K.E., Burke, E.C. et al: Hemolytic- uremic syndrome in two siblings. N. Engl. J. Med. 277: 138, 1967.PubMedCrossRefGoogle Scholar
  59. 59.
    Belobradkova, J.: The hemolytic uremic syndrome in sibs. Sb. Ved. Pr. Lek. Fak. Karlovy. Univ. (Suppl.) 20: 445, 1977.Google Scholar
  60. 60.
    Edelstein, A.O. and Tuck, S.: Familial haemolytic uraemic syndrome. Arch. Dis. Child. 53: 255, 1978.Google Scholar
  61. 61.
    Kaplan, B.S., Chesney, R.W. and Drummond, K.N.: Hemolytic uremic syndrome in families. N. Engl. J. Med. 292: 1090, 1975.PubMedCrossRefGoogle Scholar
  62. 62.
    Shapiro, H.D., Doktor, D. and Churg, J.: Thrombotic thrombocytopenic purpura (Moschcowitz1s disease): Report of a case with remission after splenectomy and steroid therapy. Ann. Intern. Med. 47: 582, 1957.PubMedGoogle Scholar
  63. 63.
    Rodriguez, J.R., Babb, D.F., Santiago, E.P. et al: Thrombotic thrombocytopenic purpura: Remission after splenectomy. N. Engl. J. Med. 257: 983, 1957.PubMedCrossRefGoogle Scholar
  64. 64.
    Hill, J.M. and Loeb, E.: Massive hormonal therapy with splenectomy in acute thrombotic thrombocytopenic purpura: Report of three cases with one eight-year cure. JAMA 173: 778, 1960.Google Scholar
  65. 65.
    Bernard, R.P., Bauman, A.W. and Schwartz, S.I.: Splen¬ectomy for thrombotic thrombocytopenic purpura. Ann. Surg. 169: 616, 1969.PubMedCrossRefGoogle Scholar
  66. 66.
    Distenfield, A. and Oppenheim, E.: The treatment of acute thrombotic thrombocytopenic purpura with corticosteroids and splenectomy. Report of three cases. Ann. Intern. Med. 65: 245, 1966.Google Scholar
  67. 67.
    Goldenfarb, P.B. and Finch, S.C.: Thrombotic thrombocytopenic purpura: A ten year survey. JAMA 226: 644, 1973.PubMedCrossRefGoogle Scholar
  68. 68.
    Reynolds, P.M., Jackson, J.M., Brine, J.A.S. et al: Thrombotic thrombocytopenic purpura - remission following splenectomy. Am. J. Med. 61: 439, 1976.PubMedCrossRefGoogle Scholar
  69. 69.
    Sacks, P.V.: Splenectomy and steroid in thrombocytopenic purpura (letter). JAMA 228: 975, 1974.PubMedCrossRefGoogle Scholar
  70. 70.
    Guttner, J.: Splenectomy, steroids, and dextran-70 in thrombotic thrombocytopenic purpura. JAMA 227: 397, 1974.CrossRefGoogle Scholar
  71. 71.
    Kadri, A., Noinuddin, M. and de Leeuw, N.K.: Phagocytosis of blood cells by splenic macrophages in thrombotic thrombocytopenic purpura. Ann. Intern. Med. 82: 799, 1975.PubMedGoogle Scholar
  72. 72.
    Amorosi, E.L. and Karpatkin, S.: Antiplatelet treatment of thrombotic thrombocytopenic purpura (editorial). Ann. Intern. Med. 86: 102, 1977.PubMedGoogle Scholar
  73. 73.
    Rubenstein, M.A., Kegan, E.M., MacGilviray, M.H. et al: Unusual remission in a case of thrombotic thrombocytopenic purpura syndrome following fresh blood exchange transfusion. Ann. Intern. Med. 51: 1419, 1959.Google Scholar
  74. 74.
    Bukowski, R.M., Hewlett, J.S., Harris, J.W. et al: Exchange transfusions in the treatment of thrombotic thrombocytopenic purpura. Semin. Hematol. 13: 219, 1976.Google Scholar
  75. 75.
    Byrnes, J.J. and Khurana, M.: Treatment of thrombotic thrombocytopenic purpura with plasma. N. Engl. J. Med. 297: 1386, 1977.PubMedCrossRefGoogle Scholar
  76. 76.
    Byrnes, J.J.: Plasma infusion in the treatment of thrombotic thombocytopenic purpura. Semin. Thromb. Hemostas. 7: 9, 1981.CrossRefGoogle Scholar
  77. 77.
    Kaplan, B.S. and Thompson, P.D.: Heparin for the hemo- lytic-uremic syndrome. N. Engl. J. Med. 294: 846, 1976.PubMedGoogle Scholar
  78. 78.
    Monnens L., Kleynen F., Van Munster, P., Schretlen, F. and Bonnerman, A.: Coagulation studies and streptokinase therapy in the haemolytic uraemic syndrome. Helv. Paediatr. Acta 27: 45, 1972.Google Scholar
  79. Stuart, J., Winterborn, M.H. and White, R.H.R.: Thrombolytic therapy in haemolytic uraemic syndrome. Br. Med. J. iii: 217, 1974.Google Scholar
  80. 80.
    Arenson, G.B. and August, C.S.: Preliminary report: Treatment of the hemolytic uremic syndrome with aspirin and dipyridamole. J. Pediatr. 86: 957, 1975.PubMedCrossRefGoogle Scholar
  81. 81.
    Thorsen, C.A., Rossi, E.C., Green, D. and Carone, F.A.: The treatment of the hemolytic-uremic syndrome with inhibitors of platelet function. Am. J. Med. 66: 711, 1979.PubMedCrossRefGoogle Scholar
  82. 82.
    Remuzzi, G., Misiani, R., Marchesi, D. et al: Treatment of the hemolytic uremic syndrome with plasma. Clin. Nephrol. 12: 279, 1979.Google Scholar
  83. 83.
    Misiani, R., Apiani, A.C., Eedefonti, A. et al: Haemolytic uremic syndrome: therapeutic effect of plasma infusion. Br. Med. J. 285: 1304, 1982.CrossRefGoogle Scholar
  84. 84.
    Lian, E.C-Y., Mui, P.T.K., Siddiqui, F.A. and Chiu, A.Y.Y.: Studies of thrombotic thrombocytopenic purpura (TTP): Purification and identity of TTP platelet aggre¬gating factor inhibitor A. Blood 60 (Suppl 1 ): 189a, 1982.Google Scholar

Copyright information

© Martinus Nijhoff Publishing, Boston 1984

Authors and Affiliations

  • J. J. Byrnes

There are no affiliations available

Personalised recommendations