Outcome of Langerhans Cell Histiocytosis Among Children

Abstract

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 , 42 patients of Langerhans cell histiocytosis were enrolled. Risk organ involvement, treatment course and outcome were analyzed.The patients were treated with vinblastine and prednisolone as first line given weekly for 6 weeks and then 3-weekly as a continuation therapy up to 6-12 months. Doses were calculated according to body surface area. 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. In our study Group A include patients who refused enucleation and opted out of recommended treatment plan and Group B patients had enucleations done and followed treatment plans. Results: Age of the patients range from< 1 to 10 years (19% <2 yrs). M: F Ratio was 2:1. Majority (72%) of the patients presented with multisystem-LCH (MS-LCH) with100% mortality in MS-LCH group (p-value=0.045) and 18/42 (43%) had risk organ involvement with 72% mortality. Twenty nine (70%) had bone lesions and 12(29%) had central nervous system involvement. Fifty five percent completed treatment, 2/42 (5%) are on treatment, 9/42 (21%) abandoned treatment and 8/42 (19%) expired due to progressive disease and worsening infection. Sixteen (38%) had reactivations of disease requiring therapy for more than one year. Twenty four percent of cases received two cycles of initiation therapy before continuation therapy started. The treatment initiated >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.