As Langerhans cells accumulate in the bone marrow, they crowd out healthy blood-forming cells, resulting in low blood counts.
As the number of oxygen-carrying red blood cells decline, patients may develop anemia, appearing pale, tired or short of breath. A decline in platelets that normally help the blood clot may lead to easy bruising or bleeding. A decline in white blood cells puts patients at increased risk for infections that can sometimes be life threatening.
LCH patients may experience a wide range of symptoms, depending on which organs and tissues have built-up deposits of Langerhans cells. These symptoms may include
- Swollen lymph nodes
- Abdominal pain
- Yellowing of the skin and whites of the eyes (jaundice)
- Delayed puberty
- Protruding eyes
- Enlargement of the spleen
- Cysts in the lungs, in more advanced stages
Roughly 1 in 50 patients with this condition suffer from impaired neurological function (neurodegeneration).
About 8 percent of patients develop one or more granulomas (small areas of inflammation) in the skull or the long bones of the arms and legs. These result in pain and swelling, and sometimes fractures.
Granulomas of the skin may cause blisters, reddish bumps, or rashes.
If the pituitary gland that produces hormones is affected, children with this condition may experience delayed onset of puberty, while older patients may experience infertility.
Pituitary damage can also lead to the production of excessive urine (diabetes insipidus) and to thyroid problems, producing changes in the patient’s metabolism, body temperature, skin and hair texture, and behavior.
Between 15 to 20 percent of patients with LCH have some form of damage to the liver or lung, as well as to the blood-forming (hematopoeitic) system.
Lung damage may involve swelling of the small airways (bronchioles) and blood vessels in the lungs, resulting in breathing problems, and increased risk of respiratory infection. Some patients develop cysts in the lung, which can sometimes cause the lungs to collapse.