Langerhans cell histiocytosis

Langerhans cell histiocytosis initially identified as an inflammatory disorder is now being referred as a rare dendritic/histiocytic neoplasm arising from dendritic cell differentiation with a heterogeneous clinical presentation manifesting in skin, bones, lungs, liver, lymph nodes, neurologic, and hematologic systems in any age group. Diagnostic work-up, staging investigations, prognostic markers, and therapeutic algorithms have seen a paradigm shift from pediatric-inspired chemotherapy regimens to a patient-tailored focused approach. Although chemotherapy is still the backbone in the era of novel circulating biomarkers and targeted agents in multisystem disease, chemoresistance and delayed neurological sequelae still remain challenges, resulting in dismal outcomes and poor quality of life.