Immunthrombozytopenie: Pathophysiologie, Diagnostik, Therapie

Zusammenfassung

Unter dem Begriff Immunthrombozytopenie (ITP) wird eine Gruppe von Erkrankungen zusammengefasst, bei denen aus unbekannten Gründen die immunologische Toleranz gegen die Thrombozyten (zeitweilig) verloren geht. Im Kindesalter wird die akute ITP häufig durch virale Infekte oder Impfungen getriggert, der Beginn der chronischen ITP wird häufig nicht wahrgenommen und die Erkrankung nimmt oft einen fluktuierenden Verlauf mit schwankenden Thrombozytenzahlen. Die primäre ITP tritt ohne (immunologische) Grund- oder Begleiterkrankungen auf, sekundäre Autoimmunthrombozytopenien werden im Zusammenhang mit SLE (systemischer Lupus erythematodes), CLL (chronische lymphatische Leukämie), Tumorerkrankungen und Infektionen beobachtet. Therapeutische Interventionen sind häufig prophylaktisch begründet, sie sollen spontane Blutungen oder Blutungen bei geplanten operativen oder diagnostischen Eingriffen vermeiden. Besonders gefürchtet sind die bei thrombozytopenischen Patienten glücklicherweise selten auftretenden spontanen zerebralen Blutungen. Neben Kortikosteroiden, immunsuppressiven Substanzen, hoch dosiertem intravenösem IgG (Immunglobulin G) spielen zunehmend die kürzlich zugelassenen Thrombopoetinrezeptoragonisten (TRA) eine Rolle bei therapierefraktären ITP-Patienten. Obwohl die Erkrankung schon lange bekannt ist und die immunologische Genese vor mehr als 60 Jahren beschrieben wurde, werden viele Aspekte der Pathogenese der ITP auch heute noch nicht völlig verstanden.

Key words

Thrombozytopenie - ITP - Immunthrombozytopenie - Blutung - Petechien - zerebrale Blutung - thrombozytäre Autoantikörper - MAIPA

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