Langerhan’s cell histiocytosis

LCH in children occurs at any age but more often in boys than girls.

The disease is not strictly cancer but it is a condition like cancer and it responds to chemotherapy. In its more serious forms LCH acts like a malignancy and therefore paediatric oncologists treat the disease.

Langerhans’ is named after Dr Paul Langerhans who first described the cells in the skin in which LCH develops and histiocytes are immune system cells found in many parts of the body.

There are two types of histiocyte cells -

• macrophage monocytes (destroy viruses, bacteria and harmful proteins)
• dendritic cells

Langerhans’ cells are dendritic cells normally found in the skin and the key airways of the body. In LCH, the Langerhans’ cells are abnormal and have spread via the bloodstream to different parts of the body – to the bone marrow, skin, lungs, lymph glands, spleen, liver and the pituitary gland. When Langerhans’ cells are present in these tissues they may cause damage.

LCH is divided into two groups:

• single-system LCH, when the disease affects only one part of the body (e.g. the skin or the bone)
• multi-system LCH, when it affects more than one part of the body.