Ocular Langerhans Cell Histiocytosis (A Multisystem Involvement of Langerhans Cell Histiocytosis)

Abstract

A 2 year old boy was referred from paediatrics OPD to Eye OPD with chief complaint of Multiple conjunctival cyst since 15 days. The patient had a prior history of recurrent chest infections. Requiring hospitalisation since 7 months of age. Upon visual acuity examination, the patient seemed to follow light.Fundus examination of both eyes were within normal limits.H& E sections of the lesions showed collection of histiocytes typical of Langerhans Cell Histiocytosis.Patient was treated with multiple cycles of Vinblastine and predinisolone as per LCH 3 guidelines. Langerhans cell histiocytosis (LCH), the most common histiocytic disorder in children. LCH lesions are granulomatous lesions consisting of pathologic “Langerhans cells” (LCs), lymphocytes (primarily T-cells), eosinophils, and macrophages. The incidence of LCH is estimated to be around 5–10 cases per million children per year and 1–2 cases per million adults per year with a male is to female ratio 1.2:1.Its pathology is ‘Misguided Myeloid Differentiation’ where state of differentiation of myeloid precursor in which activating MAPK mutation arises determines the extent and severity of disease[1]. The current standard of care for initial therapy is vinblastine/prednisone for one year.