[Updated AWMF Guideline on the Diagnosis and Treatment of Langerhans cell Histiocytosis in Children and Adolescents]. / Aktualisierte AWMF Leitlinie zur Diagnostik und Therapie der Langerhanszell Histiozytose (LCH) im Kindes- und Jugendalter.
Klin Padiatr
; 235(6): 322-330, 2023 Nov.
Article
in De
| MEDLINE
| ID: mdl-37666270
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Histiocytosis, Langerhans-Cell
Type of study:
Diagnostic_studies
/
Guideline
Limits:
Adolescent
/
Child
/
Humans
Language:
De
Journal:
Klin Padiatr
Year:
2023
Document type:
Article
Affiliation country: