Thrombozytenzerstörung bei ITP

Zusammenfassung

Die Immunthrombozytopenie (ITP) ist eine autoimmun bedingte Blutungsstörung, die durch isolierte Thrombozytopenie charakterisierte ist. Vermutlich ist die gestörte Bildung von autoreaktiven T-Zellen bei ITP Patienten für den Verlust der Selbsttoleranz gegenüber Plättchenantigenen verantwortlich. Bei ITPPatienten rufen autoreaktive T-Zellen eine unkontrollierte Proliferation von Autoantikörper bildenden B-Zellen hervor, die zu anhal-tender Autoimmunität gegen Plättchen führen. Die Autoimmunantwort bei ITP führt zu einer gesteigerten Plättchenzerstörung durch Antikörper vermittelte Phagozytose, Komplementaktivierung sowie durch T-Zell vermittelte Zytotoxizität. Zur Pathophysiologie der ITP tragen auch Anomalien in der Thrombopoese und eine durch Antikörper oder T-Zellen vermittelte Hemmung oder Zerstörung der Megakaryozyten bei. Diese Variabilität der Dysfunktionen der Effektorzellen könnte zur Heterogenität der klinischen Manifestation beitragen, aber auch zum Erfolg oder Versagen der Therapieverfahren. Das Verständnis der ITP-Pathomechanismen hilft, diagnostische und therapeutische Strategien zu optimieren.

Summary

Immune thrombocytopenia (ITP) is an auto-immune bleeding disorder characterized by isolated thrombocytopenia. A dysfunctional proliferation of autoreactive T cells is suggested to be responsible for the loss of tolerance to self-platelet antigens in ITP patients. Autoreactive T cells induce uncontrolled proliferation of autoantibody producing B cells leading to persistent anti-platelet autoimmunity in some ITP patients. The autoimmune response causes an increased destruction of platelets by antibody-mediated phagocytosis, complement activation but also by T cell mediated cytotoxicity. In addition, abnormalities in thrombopoiesis and insufficient platelet production due to antibody or T cell mediated megakaryocyte inhibition and destruction contribute to the pathophysiology of ITP. These various effector cell responses may account for the heterogeneity in the clinical manifestation of ITP and also, to success or failure of different treatment strategies. A better understanding of the mechanisms behind ITP will hopefully allow for better diagnostic and, particularly, therapeutic strategies in the future.

Schlüsselwörter

Thrombozyt - Antikörper - Thrombopenie

Keywords

Platelet - antibody - thrombocytopenia

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