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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.