Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis (LCH) is a rare disorder characterised by the abnormal accumulation of Langerhans cells, a type of immune cell normally involved in protecting the body from infection. These cells multiply excessively and form lesions that can damage bones, skin, lungs, lymph nodes, liver, spleen, or other organs. LCH can occur in both children and adults, with disease severity ranging from a single localised lesion to widespread multisystem involvement.

The exact cause is not fully understood, but many cases are linked to mutations in genes such as BRAF, suggesting that LCH behaves as an inflammatory condition with cancer-like characteristics. Symptoms depend on the organs affected and may include persistent bone pain, swollen lymph nodes, skin rashes, chronic cough, breathing difficulties, recurrent ear infections, excessive thirst, frequent urination, fatigue, and unexplained weight loss.

Diagnosis involves imaging studies such as X-rays, CT scans, MRI, or PET scans, followed by a biopsy confirming the presence of abnormal Langerhans cells. Additional blood tests and organ function assessments help determine disease extent. Treatment varies according to severity and may include surgery, corticosteroids, chemotherapy, targeted therapies for specific gene mutations, or radiation in selected cases. Early diagnosis and appropriate management significantly improve outcomes, while long-term follow-up is essential to monitor recurrence and manage potential complications.