Thrombophihe bei myeloproliferativen Syndromen

Zusammenfassung

Erworbene hämatopoetische Stammzelldefekte sind ein eindrucksvolles Beispiel für Vielfalt und Komplexität des Zustandekommens von Hämo-stasestörungen. Blutungsneigungen erklären sich durch kompliziertes Zusammenspiel von zellulären, thrombozytopenischen Zuständen mit proteolytischen Vorgängen der Hyperkoagula-bilität und Hyperfibrinolyse. Throm-boembolischen Komplikationen aber scheinen noch komplexere Mechanismen zugrunde zu liegen. Neben gesteigerter Megakaryozytenproliferation bei reaktiver Thrombozytose und entarteter Megakaryozytenproliferation bei MPD scheinen auch die anderen Blutzellen erhebliche Einflüsse auf Schweregrad und Lokalisation einer entstehenden Thrombose ausüben zu können. Das sind die Erythrozyten durch den Hämatokritwert, möglicherweise auch durch die größeren Retikulozyten, wie die Komplikationen bei Aderlaßbehandlung der PCV andeuten, und eventuell noch durch Plättchenaktivierung bei Hämolyse. Das sind aber auch die leukämischen Promyelozyten durch größenbedingte Leukostasephänomene bei ATRA-Behandlung der PML. Und da ist schließlich auch das Monozyten-Makrophagen-System, dessen Aktivierung einerseits die intestinale Lokalisation der Thrombosen bei PNH erklären könnte. Andererseits aber macht es durch Expression von Adhäsionsmolekülen und deren besonderer Rea-gibilität mit Endothel der zerebralen Strombahn die bevorzugte zentralnervöse Lokalisation der Thromboseneigung bei PNH verständlich.

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