Thrombotische Mikroangiopathie

  • P. A. Kyrle

Zusammenfassung

Die thrombotische Mikroangiopathie (TMA) umschreibt ein akutes Krankheitsbild mit hoher Letalität, bei dem es aus ungeklärter Ursache zu Thrombosierung der Mikrozirkulation und Multiorganversagen kommt. Die TMA ist durch schwere Anämie und Thrombozytopenie, uncharakteristische fluktuierende neurologische Symptome, Niereninsuffizienz und Fieber charakterisiert. Das klinische Spektrum der TMA reicht von der thrombotisch-thrombozytopenischen Purpura (TTP, Morbus Moschcowitz) mit vorwiegend neurologischer Symptomatik bis zum hämolytisch-urämischen Syndrom (HUS), bei dem klinisch die Niereninsuffizienz im Vordergrund steht.

Es soll in diesem Beitrag nicht auf das “ klassische” HUS eingegangen werden, das bei Kleinkindern im Rahmen von Infektionen mit verozytotoxinbildender Escherichia coli Serotyp 0157:H7 oder shigatoxinproduzierender Shigella dysenteriae (Typ I) auftritt und sich durch gastrointestinale Prodromalsymptomatik, Fehlen neurologischer Symptome und guter Prognose von der TMA unterscheidet.

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Literatur

  1. Amorosi EL, Ultmann JE (1966) Thrombotic thrombocytopenic purpura: a report of 16 cases and review of the literature. Medicine (Baltimore) 45:139–159Google Scholar
  2. Brain MC, Neame PB (1982) Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Thromb Hemost 8:186–197PubMedCrossRefGoogle Scholar
  3. Bukowski RM (1982) Thrombotic thrombocytopenic purpura: a review. In: Spaet TH (ed) Progress in Hemostasis and Thrombosis, vol. 6. Grune and Stratton, New York, pp 287–337Google Scholar
  4. Chen SH, Lian ECY (1989) Purification and some properties of a 59 kda platelet-aggregating protein from the plasma of a patient with thrombotic thrombocytopenic purpura. Thromb Haemostas 62:568 (Abstract)Google Scholar
  5. Cuttner J (1980) Thrombotic thrombocytopenic purpura: a ten year experience. Blood 56:302–306PubMedGoogle Scholar
  6. Gordon LI, Kwaan HC, Rossi EC (1987) Deleterious effects of platelet transfusions and recovery thrombocytosis in patients with thrombotic microangiopathy. Semin Hematol 24, 3:194–201PubMedGoogle Scholar
  7. Kaplan BS (1992) An analysis of the association of the hemolytic uremic syndrome and the birth control pill. In: Kaplan BS, Trompeter RS, Moake JL (eds) Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Marcel Dekker, New York Basel Hongkong, pp 227–240Google Scholar
  8. Kaplan BS, Kaplan P (1992) Hemolytic uremic syndrome in families. In: Kaplan BS, Trompeter RS, Moake JL (eds) Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Marcel Dekker, New York Basel Hongkong, pp 213–226Google Scholar
  9. Kelton JG, Moore J, Santos A, Sheridan D (1984) Detection of a platelet-agglutinating factor in thrombotic thrombocytopenic purpura. Ann Intern Med 101:589–593PubMedCrossRefGoogle Scholar
  10. Kennedy SS, Zacharski LR, Beck JR (1980) Thrombotic thrombocytopenic purpura; analysis of 48 unselected cases. Semin Thromb Hemost 6:341–349PubMedCrossRefGoogle Scholar
  11. Kwaan HC (1978) Clinicopathologic features of thrombotic thrombocytopenic purpura. Semin Hematol 24, 2:71–81Google Scholar
  12. Kyrle PA, Brenner B, Mannhalter C et al. (1993) Identical von Willebrand factor multimer abnormalities in idiopathic and secondary tumor-associated TTP. Thromb Haemost (Abstract) 69:949Google Scholar
  13. Moore JC, Murphy WG, Kelton JG (1989) Calpain proteolysis of von Willebrand factor enhances its binding to platelet membrane glycoprotein IIb/IIIa: an explanation for platelet aggregation in thrombotic thrombocytopenic purpura. Br J Haematol 74:457–464CrossRefGoogle Scholar
  14. Murgo AJ (1992) Cancer and chemotherapy-associated thrombotic microangiopathy. In: Kaplan BS, Trompeter RS, Moake JL (eds) Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Marcel Dekker, New York Basel Hongkong, pp 271–298Google Scholar
  15. Petitt RM (1980) Thrombotic thrombocytopenic purpura: a thirty year review. Semin Thromb Hemost 6:350–355PubMedCrossRefGoogle Scholar
  16. Remuzzi G, Rossi EC (1987) Prostacyclin in thrombotic microangiopathy. Semin Hematol 24, 2:110–118PubMedGoogle Scholar
  17. Ridolfi RL, Bell WR (1981) Thrombotic thrombocytopenic purpura. Medicine (Baltimore) 60:413–428CrossRefGoogle Scholar
  18. Rock GA, Shumak KH, Buskard NA et al. (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 325:393–397PubMedCrossRefGoogle Scholar
  19. Ruggenenti P, Remuzzi G (1990) Thrombotic thrombocytopenic purpura and related disorders. Hematology/Oncology Clinics of North America 4, 1:219–241PubMedGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 1999

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  • P. A. Kyrle

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