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LCH-­IV ­International Collaborative Treatment Protocol for Children and Adolescents with Langerhans Cell Histiocytosis

Study ID: 19926
Short Title: LCH­-IV
Trust Name: SFT,UHD,UHS
Recruitment Site: Poole Hospital,Salisbury District Hospital,Southampton General Hospital
Disease Area: CYP Haematology
Phase: N/A
Expected End Date: 01/06/2025
Postcode: SP2 8BJ
SO16 6YD
BH15 2JB
Contact Name: Amanda Pattie
Contact Email: studysupport1and3.crnwessex@nihr.ac.uk
Active: Yes

No inclusion criteria currently available

Each stratum has its own exclusion criteria. Stratum I: ­ Pregnancy (patients of child­bearing age must be appropriately tested before chemotherapy) ­ LCH­related permanent consequences (e.g. vertebra plana, sclerosing cholangitis, lung fibrosis etc.) in the absence of active disease ­ Prior systemic therapy Stratum II: ­ Patients with progressive disease in risk organs ­ Permanent consequences (e.g. sclerosing cholangitis, lung fibrosis etc.) without evidence of active LCH in the same organ or in any other locations Stratum III: ­ Isolated sclerosing cholangitis without evidence of active hepatic LCH as the only evidence of organ involvement ­ Inadequate renal function as defined by serum creatinine > 3 x normal for age. Stratum IV: ­ Pulmonary failure (requiring mechanical ventilation) not due to active LCH ­ Isolated liver sclerosis or pulmonary fibrosis, without active LCH Uncontrolled active life­threatening infection ­Decreased renal function with a GFR of < 50ml/1.73m2/min. ­Pregnancy or active breast feeding Stratum VI: ­Patients with SS­LCH who have an isolated tumorous CNS lesion (eligible for stratum V) ­Patients with isolated "CNS­risk" or multifocal bone lesions (eligible for stratum I, group 2).

Langerhans Cell Histiocytosis (LCH) is a rare disorder with highly variable clinical presentation and biological behaviours. It can affect a single system/organ (SS­LCH) or multiple systems/organs (MS­LCH). Patients with SS­LCH of the skeleton, skin or the lymph nodes have an excellent prognosis and may need no, or minimal treatment. MS­LCH is unpredictable upon diagnosis, ranging from spontaneous resolution to rapid progression and fatal outcome. Previous research has shown that combination therapy with vinblastine and prednisolone is effective for MS­LCH however more than a third of patients suffer disease reactivation. LCH patients may also suffer permanent consequences including hormone deficiencies, a neurodegenerative syndrome and lung fibrosis. This study aims to improve overall survival, reduce reactivation rates and reduce the permanent consequences. The trial is split into seven strata, designed to tailor treatment based on disease features at diagnosis and on response to treatment. Stratum I is investigating a prolongation (12 vs. 24 months) and intensification (addition of mercaptopurine) of first line therapy (vinblastine and prednisolone) via a randomisation. In stratum II, the response to a uniform initial second line therapy (prednisolone, cytarabine and vincristine) for those patients without risk organ involvement is studied following a randomised comparison of maintenance therapy with either indomethacin or mercaptopurine and methotrexate. Stratum III (cladribine/cytarabine based salvage treatment) and stratum IV (reduced intensity haemapoietic stem cell transplant) are single arm studies of second line therapy for those patients withrisk organ involvement. Stratum V explores the course and treatment of Central Nervous System­LCH (CNS­LCH). Stratum VI is an observational stratum for SS­LCH which does not require systemic treatment at diagnosis.

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