Langerhans Cell Histiocytosis

Excerpt

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder where langerin-positive cells coalesce as granulomatous lesions and deposit in various tissues throughout the body as inflammatory infiltrates. Affected organs include but are not limited to the skin, liver, gastrointestinal tract, and central nervous system (CNS) structures, such as the skull and pituitary gland. Arriving at a diagnosis of LCH requires a coordinated effort amongst an interprofessional team, beginning with a comprehensive review by a trained pathologist to perform a review of the morphologic immunohistochemical and molecular data on a sample for each individual patient with suspected LCH. Once LCh is established, providers must be aware of the differences in management between children and adults and (in adults) the difference between pulmonary and non-pulmonary LCH. Referral and long-term follow-up with an experienced hematologist are essential for the successful management of LCH.