Updated AWMF Guideline on the Diagnosis and Treatment of Langerhans cell Histiocytosis in Children and Adolescents

Klin Padiatr. 2023 Sep 4. doi: 10.1055/a-2135-3175. Online ahead of print.ABSTRACTLangerhans 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.PMID:37666270 | DOI:10.1055/a-2135-3175

Source: Hamostaseologie - September 4, 2023 Category: Hematology Authors: Thomas Lehrnbecher Martina Ahlmann Michael Albert Anke Elisabeth Barnbrock Karin Beutel Konrad Bochennek Carl Friedrich Classen Susanne Holzhauer Caroline Hutter Karoly Lakatos Roland Meisel Luciana Porto Christian Vokuhl Thomas Vraetz Milen Minkov Source Type: research