Langerhans-Zell-Histiozytose (LCH)

Zusammenfassung

Die Langerhans-Zell-Histiozytose (LCH) ist eine seltene histiozytäre Erkrankung (Inzidenz: 0,2–2,0/100 000 Kinder unter 15 Jahren) mit einem sehr heterogenen klinischen Verlauf. Spontane Regressionen werden ebenso beobachtet wie chronische Reaktivierungen. Das klinische Spektrum reicht von einem isolierten Befall des Skeletts oder der Haut, bis hin zur ausgedehnten Multiorganerkrankung mit einem progredienten und lebensbedrohli-chen Verlauf. Das diagnostische und therapeutische Vorgehen konnte durch prospektive internationale Studien der Histiocyte Society standardisiert werden. Dies resultiert in einer Verbesserung von Heilungsrate und Prognose. Je nach Krankheitsausdehnung reicht die Behandlungsstrategie von einer abwartenden Haltung oder dem Einsatz einer lokalen/topischen Therapie bei monosystemischer LCH (single system LCH; SS-LCH) bis hin zur systemischen Chemotherapie bei multi-systemischer LCH (MS-LCH). Auch auf dem Gebiet der Grundlagenforschung konnten in den letzten Jahren große Fortschritte im Hinblick auf ein besseres Verständnis der Biologie und Pathogenese der Erkrankung erzielt werden. Die dabei gewonnenen Informationen werden als Grundlage für neue Therapieansätze dienen.

Summary

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder with an incidence of 0,2–2,0/100 000 affected children under the age of 15. The disease has a wide spectrum of clinical presentations ranging from involvement of a single system (e. g. single or multiple osteolytic bone lesions or an isolated cutaneous LCH), to fulminant multisystem disease that requires intensive chemo-therapy. Single system LCH (SS-LCH) can spontaneously resolve or be cured by topical treatment, while multisystem LCH (MS-LCH) always requires systemic treatment. As a result of prospective multicenter international studies the approach to diagnosis and treatment of pediatric LCH has been standardized and the outcome has been significantly improved. The substantial progress in understanding the LCH pathobiology made in recent years gives hope for a future rational targeted treatment.

Schlüsselwörter

Langerhans-Zell-Histiozytose (LCH) - neue Behandlungsstrategien

Keywords

Langerhans cell histiocytosis (LCH) - new therapeutic targets

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