Outcome of Langerhans Cell Histiocytosis Among Children in Bangladesh Shishu Hospital and Institute, Dhaka, Bangladesh

Background: To analyze the spectrum of challenges faced in the treatment of Langerhen Cell Histiocytosis resource limited settings lacking salvage therapy. Methods: In this observational study, 30 patients of Langerhans cell histiocytosis were enrolled. Risk organ involvement, treatment course and outcome were analyzed. The patients were treated with vinblastine(iv) weekly, prednisolone(po) daily and MTX (iv) in 1st,3rd and 5th week for 6 weeks and then 6 MP daily, MTX weekly, vinblastine every 3 weekly for 6 to 12 months according to risk group. Intravenous Cytarabine pulses used for 5 days 3-weekly apart and Japanese Protocol used for refractory cases. The salvage therapy available in developed countries in the form of 2-CdA (2- chlorodeoxyadenosine) and RIC SCT reduced-intensity conditioning stem cell transplantation not available in our center. Results: Age of the patients range from< 1 to 10 years (19% 6 months after the onset of symptoms in 30/42 (72%) of cases with 100% expiries in this group and 89% of abandonment. The children presenting at younger age had risk of having MS-LCH 75% as compared to 36% in age >2years. Conclusion: Treatment abandonment, delayed diagnosis and poor social support are major challenges in treating LCH in developing countries. Early diagnosis and timely treatment with effective infection control measures and strong social support are of utmost importance to improve long term overall survival.