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LCH in Depth


Having shared my story about LCH, here is some information which you may find of interest.

Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes. There are three major classes of histiocytoses:

  • Langerhans cell histiocytosis, which is also called histiocytosis X

  • Malignant histiocytosis syndrome (now known as T-cell lymphoma)

  • Non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome)

This article focuses only on Langerhans cell histiocytosis (histiocytosis X).

Causes, incidence, and risk factors

  • Histiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.

  • Some forms of the disorder are genetic.

  • Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children ages 1 to 15. The rate peaks among children ages 5 to 10.

  • Pulmonary histiocytosis X is a specific type of this disorder that involves swelling of the small airways (bronchioles) and small blood vessels in the lungs. It is most common in adults. The inflammation leads to lung stiffening and damage.

Treatment

This disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Children may be given other medications depending on their estimated outlook. Such medications may include:

  • Cyclophosphamide

  • Etoposide

  • Methotrexate

  • Vinblastine

  • Radiation therapy or surgery may also be used to treat bone lesions.

Other treatments may include:

  • Antibiotics to fight infections

  • Breathing support (with a breathing machine)

  • Hormone replacement therapy

  • Physical therapy

  • Special shampoos for scalp problems

  • Supportive care to relieve symptoms

Expectations (prognosis)

Histiocytosis X affects many organs and can lead to death. About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function. In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.

Complications

Complications may include:

  • Diffuse interstitial pulmonary fibrosis

  • Spontaneous pneumothorax

Children may also develop:

  • Anemia caused by spreading of the tumors to the bone marrow

  • Diabetes insipidus

  • Lung problems that lead to lung failure

  • Problems with the pituitary gland that lead to growth failure

#cancer