[Therapeutic Strategies In Children And Adolescents With Langerhans Cell Histiocytosis]. / Therapiestrategien bei Kindern und Jugendlichen mit Langerhanszell Histiozytosen.

ABSTRACT

The current standard therapy for children and adolescents with newly diagnosed Langerhans cell histiocytosis (LCH) is based on the two drugs prednisone and vinblastine. In patients with insufficient treatment response or disease relapse, the choice of second-line treatment depends on risk organ involvement (liver, spleen, and hematopoietic system). This article will give an overview of current data concerning therapeutic options in the different settings of children and adolescents with LCH. Due to limited evidence, these strategies have not been described in detail in the updated guidelines on pediatric LCH. In addition, the use of targeted therapy such as MAP-kinase inhibitors will be discussed. The reference center for LCH should be contacted if therapeutic options beyond the standard regimen are considered for treatment. All children and adolescents with LCH should be enrolled in registries or prospective studies.