Langerhans cell histiocytosis in adults, explained | CION Cancer Clinics

Langerhans cell histiocytosis, or LCH, is a rare condition in which abnormal immune cells called histiocytes build up and damage tissue, most often bone, lungs, skin or the pituitary gland. It is now grouped with the blood cancers, and treatment ranges from simple watching to medicines given across the body, depending on where it is and how much of the body it affects.

What the faulty cells are

Histiocytes are immune cells that normally clear away germs and damaged tissue. In LCH, a faulty version of these cells gathers in clumps, called lesions, together with other inflammatory cells. Many of these faulty cells carry a gene change, most often in a gene called BRAF. This change is acquired during life. It is not inherited and cannot be passed to your children.

How it differs from LCH in children

LCH is better known in children, but adults get it too, and it often looks different. In adults, the lungs are affected more often, and smoking plays a large part. Adults are also more likely to have hormone problems from pituitary involvement. Because it is rare in adults, diagnosis is often delayed, and many people see several doctors before LCH is considered.

Treatment is matched to where the disease is and how much of the body it affects. Some people need very little. Others need medicines for many months.

A single bone lesion

Sometimes the biopsy itself, which scrapes out the lesion, is enough. A steroid injection into the lesion or low-dose radiotherapy may be used. Some lesions are simply watched. A lesion in the skull base or spine that threatens nearby nerves needs more active treatment.

Lung LCH

Stopping smoking completely is the most important treatment. In many people, the lung changes settle or stop getting worse after they stop. Medicines are added if the lungs keep getting worse after stopping.

Multisystem disease

Chemotherapy such as cytarabine or cladribine is commonly used. People whose lesions carry a BRAF or similar gene change may be offered targeted tablets that block that change. Targeted tablets do not suit everyone, and symptoms can return when they are stopped. Pituitary damage may need lifelong hormone replacement.

What this page cannot tell you

This page cannot tell you which treatment fits you or how your disease will behave. That depends on which organs are affected, the gene test result and your general health. At CION, the haematology team reviews each case at a tumour board and coordinates any specialised tests or medicines with qualified centres where needed. Ask your haematologist to explain your own picture.