Aktualisierte AWMF Leitlinie zur Diagnostik und Therapie der Langerhanszell Histiozytose (LCH) im Kindes- und Jugendalter

Zusammenfassung

Die Langerhanszell Histiozytose (LCH) ist eine seltene neoplastische Erkrankung, die vor allem im Kindes- und Jugendalter auftritt. Die Erkrankung kann prinzipiell jedes Organ befallen, weswegen sehr unterschiedliche klinische Erscheinungsbilder möglich sind. Der klinische Verlauf der LCH reicht von einer Spontanheilung bis hin zu einem rasch progredienten tödlichen Verlauf. Die Ausbreitungsdiagnostik bestimmt das Vorgehen. Manche Patienten qualifizieren sich für eine watch-and-wait Strategie, während andere einer Chemotherapie mit den Standardmedikamenten Vinblastin und Prednison bedürfen. Durch die Identifizierung von Mutationen im MAPK-Signalweg wächst das Interesse an zielgerichteten Medikamenten wie den BRAF-Inhibitoren. Chronisch-rezidivierende Verläufe und Spätschäden sind ein weiteres Problem der Erkrankung und stehen im Mittelpunkt derzeitiger Forschungsaktivitäten.

Abstract

Langerhans cell Histiocytosis is a rare neoplastic disease, which occurs mainly in children and adolescents. The disease may affect any organ, and therefore, the clinical symptoms vary widely. Some patients have a spontaneous remission of the disease, whereas others experience a rapid and potentially lethal clinical course. The therapeutic approach depends on the extent of the disease, and reaches from a watch-and-wait strategy to chemotherapy with the standard drugs vinblastine and prednisone. The identification of mutations in the MAPK-pathway resulted in growing interest in targeted therapy using compounds such as the BRAF inhibitors. Chronic relapses and permanent sequelae are important problems of LCH and are the focus of current research.

Schlüsselwörter

Leitlinie - Langerhanszell Histiozytose - Kind - Therapie

Key words

guideline - Langerhans cell histiozytosis - child - therapy

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