Title

A multicenter, retrospective study on demography, presentation, treatment, and outcome of Langerhans cell histiocytosis in India.
● Principal Investigator: Dr Ramya Uppuluri


Aim

● To analyse the demography, presentation, treatment, outcome in childhood LCH in India


Eligibility Criteria

● Inclusion Criteria

● Children up to 18 years of age diagnosed with biopsy proven LCH except for isolated CNS LCH where biopsy is not feasible
● Children receiving primary treatment in the participating centre

● Exclusion Criteria:

● Sample size Records not available



● Outcome Measures to be evaluated:

● To obtain data on demography, presentataion (Clinical, imaging, laboratorty) in childhood LCH
● To obtain data on treatment provided for childhood LCH
● To analyse overall survival and event free survival in childhood LCH


Summary

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm arising from clonal expansion of myeloid precursors, with underlying activating mutations in MAPK pathway, and characterized by CD1a/CD207 positive Langerhans cells. The presentation can be varied from involvement of single site bone to multifocal bone disease, to multisystem disease including risk organ involvement namely hematopoietic system, liver, and spleen. Treatment is determined by extent of disease involvement within 12 months treatment duration with Vinblastine/steroids being standard of care. Evidence also supports local treatment for single site bone lesions. Published long term overall survival for high-risk disease is >85%, and for low-risk disease is >95%. Disease reactivation and requiring second line therapy. Several single Centre studies from India have reported survival rates as above in multisystem and single site LCH.
With the above background, we aim to perform a multicenter study in India with the aim to develop a comprehensive database on demography, presentation, treatment and outcome of LCH in India. This will pave the way for a prospective LCH registry and help further identify areas of improvement and development to provide optimal outcome and survival in childhood LCH.