Overview of Langerhans Cell Histiocytosis among Children

Abstract

Aim: To analyze clinical manifestations, course and outcome of Langerhans Cell Histiocytosis in children in resource limited settings lacking salvage therapy. Study design: Observational retrospective study Place and duration of study: Department of Haematology/Oncology, The Children’s Hospital, Lahore Pakistan from 1st January 2011 to 31st December 2018. Methodology: Sixty-five patients with age range from<1 to 8 years included analysing their age, gender, clinical classification, course of therapy and outcome. The major treatment was composed of either prednisolone and vinblastine or cytarabine pulses. Results: There were 59% males and 41% females. Forty-seven (72%) patients presented with multi system-LCH with 49% Risk Organ involvement. Most of them 42 (65%) had bone lesions while 15patients (23%) presented with central nervous system involvement. Forty patients (61%) have completed treatment, 11(17%) left against medical advice and 12(18%)patients expired due to progressive disease and worsening infection. Only 2 patients were put on palliation with progressive brain parenchyma disease. 22 patients (34%) had reactivations of disease requiring therapy for more than one-year (p-value=0.06), while 15 (23%) patients received two cycles of initiation therapy before continuation therapy started. The treatment initiated >6 months after the onset of symptoms in 48 (74%) patients. Conclusion: Early diagnosis and timely initiation of therapy are of utmost importance to reduce mortality and morbidity. There is a dire need of social support to reduce treatment abandonment in low-middle-income countries LMIC. Keywords: Paediatric Langerhans cell histiocytosis, Resource-limited settings, Delayed diagnosis, Abandonment