LCH-IV: (NACHO) INTERNATIONAL COLLABORATIVE TREATMENT PROTOCOL FOR CHILDREN AND ADOLESCENTS WITH LANGERHANS CELL HISTIOCYTOSIS
Study ID: STU-2018-0071
Systemic therapy is indicated for disease control in all patients with MS-LCH and for a certain subgroup of patients with SS-LCH (isolated CnS-risk lesion or multifocal bone lesions). The combination of steroids and vinblastine is the standard first-line combination for patients needing systemic therapy.
Liver; Bones and Joints; Brain and Nervous System; Lung/Thoracic; Other Hematopoietic
Primary objective: -To investigate whether mortality in MS-LCH can be further decreased by an early switch of patients with risk organ involvement who do not respond to front-line therapy to more intensive salvage treatment -To investigate in a randomized fashion whether further prolongation and intensification of continuation therapy will reduce the reactivation rate and permanent consequences in MS-LCH. -To investigate in a randomized fashion whether prolongation of continuation therapy (6 vs. 12 months) will reduce the reactivation rate and permanent consequences in SS-LCH patients with isolated CnS-Risk lesion or multifocal bone lesions. -To investigate whether second-line therapy with PReD or aRa-C or VCR for 24 weeks, followed by 24 months of continuation therapy (indometacin vs. 6-MP or MTX) can help achieve disease resolution, prevent further reactivations and permanent consequences in patients with non-risk LCH (MS-LCH without risk organ involvement, isolate[See protocol for complete text]