Pathophysiologie und Diagnostik der Immunthrombozytopenie

Zusammenfassung

Die Immunthrombozytopenie (ITP) entsteht durch Autoantikörper-Bildung gegen Oberflächenantigene der Blutplättchen. Die Erkrankung tritt als primäre ITP ohne eindeutigen Auslöser oder als sekundäre ITP (Medikamente, andere Krankheitsbilder) auf. Die Autoantikörper-Bildung führt zu einem Verlust von Thrombozyten in Milz oder Leber und reduziert daneben auch die Bildung von Thrombozyten. Die Synthese von Thrombopoetin ist bei ITP-Patienten – im Gegensatz anderen Bildungsstörungen der Thrombozyten – reduziert. Die ITP ist eine Ausschlussdiagnose, beweisende Laboruntersuchung liegen nicht vor. Nur in 50% der Fälle lassen sich Glykoprotein-spezifische Antikörper nachweisen. Von besonderer Bedeutung ist der Ausschluss der EDTA-induzierten Pseudothrombozytopenie. Andere Ursachen der Thrombozytopenie sollten durch (Medikamenten-)Anamnese, körperliche Untersuchung und gegebenenfalls Knochenmarkpunktion ausgeschlossen werden.

Abstract

Immune thrombocytopenia (ITP) is due to autoantibodies against platelet surface antigens. ITP is considered as either primary, with no clear etiology, or as secondary ITP (drug-induced; underlying diseases). Autoantibodies lead both to loss of platelets in the spleen and/or liver but simultaneously reduce their production. Contrary to other disorders with thrombocytopenia, ITP has reduced levels of thrombopoetin. ITP remains a diagnosis of exclusion. A single defining laboratory test does not exist. Glycoprotein-specific antibodies can be detected in only about 50% of cases. Ruling out EDTA-induced pseudo thrombocytopenia is of particular relevance. Secondary causes of thrombocytopenia should be excluded through medical history (especially medication history), physical examination and possibly bone-marrow puncture.

Kernaussagen

• Die Immunthrombozytopenie ist eine Autoimmun-Erkrankung, die mit einem erhöhten Abbau von Thrombozyten und gleichzeitig verminderter Produktion von Thrombozyten einhergeht.

• Der Ausschluss einer Pseudothrombozytopenie sollte bei Fehlen eindeutiger Blutungszeichen initial immer erfolgen.

• Die Letalität der ITP steigt in höherem Lebensalter stark an.

• Ein beweisender Test, der die Diagnose ITP absichert, existiert nicht.

Schlüsselwörter

Immunthrombozytopenie - Thrombopoetin - Desialylierung - Pathophysiologie - Diagnose

Keywords

immune thrombocytopenia - pathophysiology - diagnostics

Publication History

Received: 05 November 2023

Accepted after revision: 30 April 2024

Article published online:
01 July 2024

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