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19-05-2023 | Langerhans Cell Histiocytosis | Review Article

Treatment of Langerhans Cell Histiocytosis and Histiocytic Disorders: A Focus on MAPK Pathway Inhibitors

Authors: Ashley V. Geerlinks, Oussama Abla

Published in: Pediatric Drugs | Issue 4/2023

Abstract

Histiocytic disorders are rare diseases defined by the clonal accumulation of a macrophage or dendritic cell origin. These disorders include Langerhans cell histiocytosis, Erdheim–Chester disease, juvenile xanthogranuloma, malignant histiocytoses, and Rosai–Dorfman–Destombes disease. These histiocytic disorders are a diverse group of disorders with different presentations, management, and prognosis. This review focuses on these histiocytic disorders and the role of pathological ERK signaling due to somatic mutations in the mitogen-activated protein kinase (MAPK) pathway. Over the last decade, there has been growing awareness of the MAPK pathway being a key driver in many histiocytic disorders, which has led to successful treatment with targeted therapies, in particular, BRAF inhibitors and MEK inhibitors.

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Title: Treatment of Langerhans Cell Histiocytosis and Histiocytic Disorders: A Focus on MAPK Pathway Inhibitors
Authors: Ashley V. Geerlinks
Oussama Abla
Publication date: 19-05-2023
Publisher: Springer International Publishing
Keywords: Langerhans Cell Histiocytosis
Xanthogranuloma
Histiocytosis
Targeted Therapy
Published in: Pediatric Drugs / Issue 4/2023
Print ISSN: 1174-5878
Electronic ISSN: 1179-2019
DOI: https://doi.org/10.1007/s40272-023-00569-8

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Treatment of Langerhans Cell Histiocytosis and Histiocytic Disorders: A Focus on MAPK Pathway Inhibitors

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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