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Thrombotisch-thrombozytopenische Purpura (TTP)

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Definition:
Thrombotische Mikroangiopathie mit mikroangiopathischer hämolytischer Anämie, Thrombozytopenie und disseminierten mikrovaskulären Thromben. Zugrunde liegender Pathomechanismus ist ein Mangel an ADAMTS13, einer Protease, die spezifisch für die Spaltung des Von-Willebrand-Faktors zuständig ist.
Häufigkeit:
Sehr selten, jährliche Inzidenz in Deutschland ca. 1,5/1.000.000.
Symptome:
Häufig unspezifisch. Abgeschlagenheit, Luftnot, neurologische Symptome, Ischämiezeichen, Nierenversagen, Hauteinblutungen, Petechien.
Diagnostik:
Hämolyse-Parameter (erhöhte LDH, nicht nachweisbares Haptoglobin, erhöhte Retikulozytenzahl), Schistozyten im Blutausstrich, deutlich verringerte ADAMTS13-Aktivität (< 10 %).
Therapie:
Sofortige Einleitung einer Plasmapherese, Gabe von Fresh Frozen Plasma (FFP) und Kortikosteroiden. Erweiterung der Therapie durch Hinzunahme von Rituximab und Caplacizumab.
Prognose:
Unbehandelt liegt die Letalität bei > 90 %, unter Therapie bei ca. 5–10 %.
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  • Kristin Haavisto, Dr. med., Fachärztin für Innere Medizin und Hämato-/Onkologie, Münster