Therapiestrategien bei Kindern und Jugendlichen mit Langerhanszell Histiozytosen

Zusammenfassung

Die derzeitige Standardtherapie bei Kindern und Jugendlichen mit neu diagnostizierter Langerhanszell Histiozytose (LCH) beruht auf den Medikamenten Prednison und Vinblastin. Im Falle eines schlechten Therapieansprechens bzw. Reaktivierung hängt das weitere Vorgehen von Faktoren wie zum Beispiel des Befalls eines Risikoorgans wie Leber, Milz oder hämatopoetisches System ab. Der folgende Artikel gibt eine Übersicht über die wichtigsten aktuellen Daten zu Therapieoptionen in den unterschiedlichen klinischen Situationen, die aufgrund der fehlenden Evidenz meist nicht ausführlich in den aktualisierten Leitlinien dargestellt werden. Auch werden neue Therapieoptionen wie Inhibitoren des MAP-Kinase Signalweges diskutiert. Falls diese Therapiestrategien in Erwägung gezogen werden, sollte dies mit dem zuständigen Referenzzentrum diskutiert werden. Zudem sollten alle Kinder und Jugendliche mit LCH in laufende Register oder Studien eingeschlossen werden.

Abstract

The current standard therapy for children and adolescents with newly diagnosed Langerhans cell histiocytosis (LCH) is based on the two drugs prednisone and vinblastine. In patients with insufficient treatment response or disease relapse, the choice of second-line treatment depends on risk organ involvement (liver, spleen, and hematopoietic system). This article will give an overview of current data concerning therapeutic options in the different settings of children and adolescents with LCH. Due to limited evidence, these strategies have not been described in detail in the updated guidelines on pediatric LCH. In addition, the use of targeted therapy such as MAP-kinase inhibitors will be discussed. The reference center for LCH should be contacted if therapeutic options beyond the standard regimen are considered for treatment. All children and adolescents with LCH should be enrolled in registries or prospective studies.

Schlüsselwörter

Langerhanszell Histiozytose - Kind - Therapiestrategie - Stamzelltransplantation - zielgerichtete Therapie

Key words

Langerhans cell histiocytosis - child - therapeutic options - hematopoietic cell transplantation - targeted therapy

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