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Essenzielle Thrombozythämie

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Definition:
Myeloproliferative Neoplasie mit dauerhaft erhöhter Thrombozytenzahl im Blut durch eine monoklonale autonome Vermehrung der myeloischen Stammzelle. 
Häufigkeit:
 1–2,5/100.000/Jahr, Altersgipfel 50–70 Jahre.
Symptome:
Häufig asymptomatisch. Mirkozirkulationsstörungen mit Kopfschmerzen, Sehstörungen und Erythromelalgie (schmerzhafte Rötung von Händen und Füßen), venöse und arterielle thrombotische Ereignisse, Blutungsneigung.
Befunde:
Oft blande; gelegentlich Hepatosplenomegalie, Erythromelalgie, Thrombosezeichen.
Diagnostik:
Zunächst Ausschluss einer sekundären Thrombozythämie durch Anamnese, Labor- und apparative Diagnostik. Beweisend sind Gendiagnostik und Knochenmarkshistologie.
Therapie:
Keine kausale Therapie möglich. Reduktion von thrombotischen Komplikationen durch Thrombozytenaggregationshemmung mit ASS und zytoreduktive Therapie mit Hydroxyurea. 
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  • Dietrich August, Dr. med., Arzt, Freiburg im Breisgau