OBJECTIVES: To determine the profile and outcome of patients with Langerhans Cell Histiocytosis admitted at National Children’s Hospital.
METHODOLOGY: This retrospective descriptive study included 15 patients admitted at National Children’s Hospital and managed as Langerhans cell histiocytosis (LCH) from 2007 to 2017. Medical charts were reviewed and data retrieved with regards to age, sex, age of diagnosis, common presenting signs and symptoms, site of involvement, organ dysfunction, diagnostics done, treatment and the outcome of the patient whether survive, died, lost to follow up or ongoing treatment within 1 year after diagnosis.
RESULTS: 15 patients were included in this study with 60% within the age range 1 to 4 years old with male predominance of 2:1. 11 patients (73.33%) were diagnosed between the age of 1 to 4 years old. Majority of patients presented with head mass and/or pallor at 46.67%, followed by presence of cervical lymphadenopathy or rashes at 26.67%. LCH affects many organ systems, but majority of cases involved bones at 60% and 40% of patients had bone marrow dysfunction. Liver dysfunction was present in 27%. 60% of the patients
underwent skin biopsy with immunohistochemical staining. For 27% of the patients, it was noted that alkaline phosphatase had elevated results. Out of the 15 patients, 46.67% died, 6 patients were lost to follow up. 40% of the patients underwent chemotherapy and 2 completed the regimen and were cured.
CONCLUSIONS: This study showed that majority of LCH patients are male, with the most common presenting symptoms of head mass and pallor. Majority of the patients developed bone involvement and bone marrow dysfunction. 60% of the patients underwent biopsy with immunohistochemical staining and 46.67% of the patients died within 1 year of diagnosis. With the use of clinical grounds and radiological imaging we can make a diagnosis earlier and initiate treatment.