Childhood Langerhans Cell Histiocytosis: Experience from a Tertiary Care Hospital in Eastern India

Introduction
Langerhans cell histiocytosis is a rare disorder characterized by the abnormal proliferation and accumulation of Langerhans cells, with clinical presentations ranging from localized bone lesions to life-threatening multisystem disease. Diagnosis is established based on clinical findings and positive immunohistochemical staining for CD1a and/or Langerin (CD207).

Objective
To study the clinico-epidemiological profile and treatment outcomes of children (< 18 years) with Langerhans cell histiocytosis.

Material and methods
A retrospective analysis of 20 pediatric cases of Langerhans cell histiocytosis was performed. All patients were diagnosed and treated according to the Histiocyte Society Evaluation and Treatment Guidelines published in 2009. The study was conducted over a 3-year period.

Results
The median age at diagnosis was 3 years, with a male-to-female ratio of 3 : 1. Failure to thrive was the most common presenting symptom, observed in 70% of patients. Multisystem involvement was present in 75% of cases, with skeletal involvement observed in all patients and cutaneous involvement in 50%. Risk organ involvement was identified in 86.6% of multisystem cases, predominantly affecting the liver in 73.3%. Special site involvement included the ear in 15% and the central nervous system in 10% of patients. Laboratory abnormalities included leukopenia in 15%, anemia in 20%, thrombocytopenia in 20%, elevated alkaline phosphatase levels in 50%, and transaminitis in 35%. All patients received 6 weeks of treatment with vinblastine and prednisolone, with subsequent therapy determined by treatment response. Following initial therapy, 80% of patients showed improvement, while 20% experienced disease progression and required salvage therapy. One-year overall survival was 100%.

Conclusions
Langerhans cell histiocytosis is a rare, distinct, and highly heterogeneous condition with diverse clinical manifestations. Early diagnosis is crucial for timely initiation of treatment and prevention of disease progression. Vinblastine and prednisolone represent an effective treatment option, leading to improved response rates and overall survival.