Ocular Langerhans Cell Histiocytosis (A Multisystem Involvement of Langerhans Cell Histiocytosis)
Keywords:Cell, Conjunctival, Patient.
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. The current standard of care for initial therapy is vinblastine/prednisone for one year.
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Copyright (c) 2023 Anjali Sinha, Abhishek Kumar Sinha, Himanshu Shekhar
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