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Thrombocytopenia in Hemolytic-Uremic Syndrome

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Acute Renal Disorders and Renal Emergencies

Abstract

Thrombocytopenia, unlike acute hemolysis and acute nephropathy, does not occur in every patient with hemolytic uremic syndrome (HUS). The mechanism whereby thrombocytopenia occurs is not known, the pathogenetic role of platelets in HUS has not been clearly defined, the duration of thrombocytopenia is variable and the level to which the platelets fall has no prognostic value (1–3). Thrombocytopenia, however, is such a dramatic laboratory observation that much attention has been directed at this finding and, although the role of platelets in the pathogenesis of HUS has been unclear, a variety of therapeutic agents has been advocated on the assumption that normalization of the platelet count may be beneficial. None, unfortunately, has of value and enthusiasm has waned for the use of heparin, aspirin and dipyridamol (4–11).

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References

  1. Katz, J., Krawitz, S., Sacks, D.V., et al. Platelet, erythrocyte, and fibrinogen kinetics in the hemolytic-uremic syndrome of infancy. J. Pediatr., 83: 739, 1973.

    Article  PubMed  CAS  Google Scholar 

  2. Metz, J.: Observations on the mechanisms of the haematological changes in the haemolytic uraemic syndrome of infancy. Br. J. Haematol., 23 (Suppl.): 53, 1972.

    Article  PubMed  Google Scholar 

  3. Rossi, E.C., Carone, F.A., and del Greco, F.: Platelets and the hemolytic uremic syndrome. Ann. Clin. Lab. Sci., 11: 269, 1981.

    PubMed  CAS  Google Scholar 

  4. Fong, J.S.C., de Chadarevian, J-P., and Kaplan, B.S.: Hemolytic- uremic syndrome: Current concepts and management. Ped. Clin. N. Amer., 29: 835, 1982.

    CAS  Google Scholar 

  5. Proesmans, W., Ki-Muaka, B., Van Damme, B., et al.: The use of heparin in childhood hemolytic-uremic syndrome. In Remuzzi, G., Mecca, G., and de Gaetano, G. (eds.): Hemostasis,Prostaglandins and Renal Disease, New York, Raven Press, 1980.

    Google Scholar 

  6. Monnens, L., van Collenburg, J., de Jog, M., et al.: Treatment of the hemolytic-uremic syndrome. Helv. Paediatr. Acta, 33: 321, 1978.

    PubMed  CAS  Google Scholar 

  7. O’Regan, S., Chesney, R.W., Mongeau, J-G., et al.: Aspirin and dipyridamole therapy in the hemolytic-uremic syndrome. J. Pediatr., 97: 473, 1980.

    Article  PubMed  Google Scholar 

  8. Vitacco, M., Avalos, J.S., and Gianantonio, C.A.: Heparin therapy in the hemolytic-uremic syndrome. J. Pediatr., 83: 271, 1973.

    PubMed  CAS  Google Scholar 

  9. 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.

    Article  PubMed  CAS  Google Scholar 

  10. Powell, H.R., and Ekert, H.: Streptokinase and anti-thrombotic therapy in the hemolytic-uremic syndrome. J. Pediatr., 84: 345, 1974.

    Article  PubMed  CAS  Google Scholar 

  11. Stuart, J., Winterborn, M.H., White, R.H.R., and Flinn, R.M.: Thrombolytic therapy in haemolytic-uraemic syndrome. Br. Med. J., 3: 217, 1974.

    Article  PubMed  CAS  Google Scholar 

  12. Remuzzi, G., Misiani, R., Marchesi, D., et al.: Treatment of the hemolytic-uremic syndrome with plasma. Clin. Nephrol., 12: 279, 1979.

    CAS  Google Scholar 

  13. Remuzzi, G., Misiani, R., Marchesi, D., et al.: Haemolytic-uraemic syndrome: Deficiency of plasma factor(s) regulating prostacyclin activity? Lancet, ii: 871, 1978.

    Google Scholar 

  14. Remuzzi, G., Marchesi, D., Misiani, R., et al.: Familial deficiency of a plasma factor stimulating vascular prostacyclin activity. Thromb. Res., 16: 517, 1979.

    Article  PubMed  CAS  Google Scholar 

  15. Defreyn, G., Proesmans, W., Machin, S.J., Lemmens, F., and Vermylen, J.: Abnormal prostacyclin metabolism in the hemolytic uremic syndrome: equivocal effect of prostacyclin infusions. Clin. Nephrol., 18: 43, 1982.

    PubMed  CAS  Google Scholar 

  16. Harker, L.A., and Slichter, S.J.: Platelet and fibrinogen consumption in man. N. Engl. j. Med., 287: 999, 1972.

    Article  PubMed  CAS  Google Scholar 

  17. Miller, K., Dresner, I.G., and Michael, A.F.: Localization of platelet antigens in human kidney disease. Kidney Int., 18: 472, 1980.

    Article  PubMed  CAS  Google Scholar 

  18. Calvert, G.D.: Post-partum haemolytic uraemic syndrome: Case report and brief review. J. Obstet. Gynaecol. Br. Commonw., 79: 244, 1972.

    Article  PubMed  CAS  Google Scholar 

  19. Hall, G.H., Caldwell, R.A., and Shaldon, C.: Heparin-treated haemolytic-uraemic syndrome simulating Fabry’s disease. Br. Med. J., 2: 317, 1971.

    Article  PubMed  CAS  Google Scholar 

  20. van Wieringen, P.M.V.: Het Haemolytisch-Uraemisch Syndroom op de Kinderleeftijd. Thesis., 1977. Drukkerij van Mameren B.V. - Nijmegen.

    Google Scholar 

  21. Hoyer, J.R., Michael, A.F., and Hoyer, L.W.: Immunofluorescent localization of antihemophilic factor antigen and fibrinogen in human renal disease. J. Clin. Invest., 53: 1375, 1974.

    Article  PubMed  CAS  Google Scholar 

  22. Jaffe, E.A.: Endothelial cells and the biology of factor VIII. N. Engl. J. Med., 296: 377, 1977.

    Article  PubMed  CAS  Google Scholar 

  23. Ekberg, M., Nilsson, J.M., and Denneberg, T.: Coagulation studies in hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Acta Med. Scand., 196: 373, 1974.

    Article  PubMed  CAS  Google Scholar 

  24. Appiani, A.C., Edefonti, A., Bettinelli, A., Cossu, M.M., Paracchini, M.L., and Rossi, E.: The relationship between plasma levels of the factor VIII complex and platelet release products (B-thromboglobulin and platelet factor 4) in children with hemolytic-uremic syndrome. Clin. Nephrol., 17: 195, 1982.

    PubMed  CAS  Google Scholar 

  25. Moncada, S., and Amezcua, J.L.: Prostacyclin, thromboxane A2 interactions in haemostasis and thrombosis. Haemostasis, 8: 252, 1979. Gingrich, R.D., and Hoak, J.C.: Platelet-endothelial cell interactions. Sem. Hemat., 16: 208, 1979.

    Google Scholar 

  26. Harker, L.A., Ross, R., and Glosmet, J.A.: The role of endothelial cell injury and platelet response in atherogenesis. Thrombos. Haemostas., 39: 312, 1978.

    CAS  Google Scholar 

  27. Born, G.V.R., and Wehmeier, A.: Inhibition of platelet thrombus formation by chlorpromazine acting to diminish haemolysis. Nature, 282: 212, 1979.

    Article  PubMed  CAS  Google Scholar 

  28. Avalos, J.S., Vitacco, M., Penalver, J., et al.: Coagulation studies in the hemolytic-uremic syndrome. J. Pediatr., 76: 538, 1970.

    Article  PubMed  CAS  Google Scholar 

  29. Montgomery, R.R., and Hathaway, W.E.: The hemolytic-uremic syndrome. In Mcintosh, R.M., Guggenheim, S.J., and Schrier, R.W. (eds.): Kidney Disease: Hematologic and Vascular Problems, New York, John Wiley and Sons, 1977.

    Google Scholar 

  30. Monnens, L., van Aken, w., and de Jong, M.: “Active” intravascular coagulation in the epidemic form of hemolytic-uremic syndrome. Clin. Nephrol., 17: 284, 1982.

    PubMed  CAS  Google Scholar 

  31. Brandt, P., Jespersen, J., and Gregersen, G.: Post-partum haemolytic- uraemic syndrome treated with antithrombin-III. Nephron, 27: 15, 1981.

    Article  PubMed  CAS  Google Scholar 

  32. Wessler, S., and Gitel, S.N.: Heparin: New concepts relevant to clinical use. Blood, 53: 525, 1979.

    PubMed  CAS  Google Scholar 

  33. Remuzzi, G., Livio, M., Mecca, G., et al.: Prostacyclin and kidney disease: Focus on uremia and thrombotic microangiopathy. In Lewis, P.J., and O’Grady, J. (eds.): Clinical Pharmacology of Prostacyclin, New York, Raven Press, 1981.

    Google Scholar 

  34. Wiles, P.G., Solomon, L.R., Lawler, W., and Mallick, N.P.: Inherited plasma factor deficiency in haemolytic-uraemic syndrome. Lancet, i: 1105, 1981.

    Google Scholar 

  35. Dormandy, T.L.: Free-radical oxidation and antioxidants. Lancet, i: 647, 1978.

    Google Scholar 

  36. Ali, M., Gudbranson, C.G., and McDonald, W.D.: Inhibition of human cyclooxygenase by alpha-tocopherol. Postaglandins Med., 4: 79, 1980.

    Article  CAS  Google Scholar 

  37. Fong, J.S.C.: Alpha-tocopherol: Its inhibition on human platelet aggregation. Experientia, 32: 639, 1976.

    Article  PubMed  CAS  Google Scholar 

  38. Panganamala, R.V., Miller, J.S., Gwebu, E.T., et al.: Differential inhibitory effects of Vitamin E and other antioxidants on prostaglandin synthetase, platelet aggregation and lipoxidase. Prostaglandins, 14: 261, 1977.

    CAS  Google Scholar 

  39. O’Regan, S., Chesney, R.W., Kaplan, B.S., et al.: Red cell membrane abnormalities in the hemolytic-uremic syndrome. Clin. Nephrol. 15: 14, 1980.

    Google Scholar 

  40. Horowitz, H.T., Stein, I.M., Cohen, B.D., and White, J.G.: Further studies on the platelet inhibitory effect of guanidinosuccinic acid and its role in uremic bleeding. Am. J. Med., 49: 336, 1970.

    Article  PubMed  CAS  Google Scholar 

  41. Rabiner, S.F., and Moiinas, F.: The role of phenol and phenolic acids on the thrombocytopathy and defective platelet aggregation of patients with renal failure. Am. J. Med., 49: 346, 1970.

    Article  PubMed  CAS  Google Scholar 

  42. Beattie, T.J., Murphy, A.V., Willoughby, M.L.N., et al.: Plasmapheresis in the haemolytic- raemic syndrome in children. Br. Med. J., 282: 1667, 1981.

    Article  CAS  Google Scholar 

  43. Lian, E.C., Harkness, D.R., Byrnes, J.J., Wallach, H., and Nunez, R.: Presence of a platelet aggregating factor in the plasma of patients with thrombotic thrombocytopenic purpura (TTP) and its inhibition by normal plasma. Blood, 53: 333, 1979.

    PubMed  CAS  Google Scholar 

  44. Upshaw, Jr., J.D.: Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N. Engl. J. Med., 298: 1350, 1978.

    Article  PubMed  Google Scholar 

  45. Fry, G.L., Czervionke, R.L., Hoak, J.L., Smith, J.B., and Haycraft, D.L.: Platelet adherence to cultured vascular cells: influence of prostacyclin (PGI2). Blood, 55: 271, 1980.

    PubMed  CAS  Google Scholar 

  46. Edenfonti, A., Tentori, F., Bertinelli, A., et al.: Pattern of platelet serontonin (5HT), plasma beta-thromboglobulin (BTG) and platelet factor 4 (PF4) in hemolytic uremic syndrome (HUS) in children. Int. J. Pediatr. Nephrol., 2: 149, 1981.

    Google Scholar 

  47. Beattie, T.J., Murphy, A.V., and Arneil, G.C.: Studies on prostaglandin metabolism in hemolytic-uremic syndrome. Int. J. Pediatr. Nephrol., 2: 130, 1981.

    Google Scholar 

  48. Fong, J.S.C., and Kaplan, B.S.: Impairment of platelet aggregation in hemolytic uremic syndrome: Evidence for platelet “exhaustion”. Blood, 60: 564, 1982.

    PubMed  CAS  Google Scholar 

  49. Green, D., Santhanam, S., Krumlovsky, F.A., et al.: Elevated B- thromboglobulin in patients with chronic renal failure: Effect of hemodialysis. J. Lab. Clin. Med., 95: 679, 1980.

    PubMed  CAS  Google Scholar 

  50. Hope, W., Martin, T.J., Chesterman, C.N., and Morgan, F.J.: Human B-thromboglobulin inhibits PGI2 production and binds to a specific site in bovine aortic endothelial cells. Nature, 282: 210, 1979.

    Article  PubMed  CAS  Google Scholar 

  51. Parbtani, A., and Cameron, J.S.: Platelet and plasma serotonin concentrations in glomerulonephritis I. Thromb. Res., 15: 109, 1979.

    Article  PubMed  CAS  Google Scholar 

  52. Pareti, F.I., Capitanio, A., Mannucci, L., et ah: Acquired dysfunction due to the circulation of “exhausted” platelets. Am. J. Med., 69: 235, 1980.

    Article  PubMed  CAS  Google Scholar 

  53. Kaplan, B.S., and Fong, J.S.C.: Platelet aggregation in haemolytic uraemic syndrome. Lancet, 1: 46, 1980.

    Article  PubMed  CAS  Google Scholar 

  54. Kaplan, B.S., and Fong, J.S.C.: Reduced platelet aggregation in the hemolytic uremic syndrome. Thromb. Haemost., 43: 154, 1980.

    PubMed  CAS  Google Scholar 

  55. Mendoza,S.A.,Sellers, B.B., and Griswold, W.R.: Hemolytic uremic syndrome and idiopathic thrombocytopenic purpura in a single patient. J. Pediatr., 91: 851, 1977.

    Google Scholar 

  56. Kaplan, B.S.: Hemolytic-uremic syndrome followed by idiopathic thrombocytopenic purpura. Int. J. Pediatr. Nephrol. 2: 97, 1981.

    PubMed  CAS  Google Scholar 

  57. This is publication number 00000 of The McGill University-Montreal Children’s Hospital Research Institute.

    Google Scholar 

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© 1984 Martinus Nijhoff Publishing, Boston

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Fong, J.S.C., Kaplan, B.S. (1984). Thrombocytopenia in Hemolytic-Uremic Syndrome. In: Strauss, J. (eds) Acute Renal Disorders and Renal Emergencies. Developments in Nephrology, vol 7. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-3822-2_3

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  • DOI: https://doi.org/10.1007/978-1-4613-3822-2_3

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