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Histiocytosis X is a group of disorders (Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma) in which abnormal scavenger cells called histiocytes and another immune system cell type called eosinophils proliferate, especially in the bone and lung, often causing scars to form.
Letterer-Siwe disease starts before age 3 and is usually fatal without treatment. The histiocytes damage not only the lungs but also the skin, lymph glands, bone, liver, and spleen. Collapse of a lung (pneumothorax) may occur.
Hand-Schüller-Christian disease usually begins in early childhood but can start in late middle age. The lungs and bones are most frequently affected. Rarely, damage to the pituitary gland causes bulging eyes (exophthalmos) and diabetes insipidus, a condition in which large quantities of urine are produced, leading to dehydration.
Eosinophilic granuloma tends to start between ages 20 and 40. It usually affects the bones but also affects the lungs in 20% of people; sometimes only the lungs are involved. When the lungs are affected, the symptoms can include coughing, shortness of breath, fever, and weight loss, but some people have no symptoms. Collapse of a lung (pneumothorax) is a common complication.
Treatment & Prevention
All three disorders may be treated with corticosteroids and cytotoxic drugs such as cyclophosphamide, although no therapy is clearly beneficial. The therapy for bone involvement is similar to that for bone tumors.
Prognosis; Complications
People with Hand-Schüller-Christian disease or eosinophilic granuloma may recover spontaneously.
Death usually results from respiratory failure or heart failure.
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Signs, symptoms & indicators of Histiocytosis X (Rare):
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GLOSSARY
Corticosteroid (Corticosteroids)
Steroid hormone produced by the adrenal cortex.
Cytotoxic
A compound that produces a toxic effect on cells.
Diabetes Insipidus
Excessive production of urine, usually due to insufficient production of antidiuretic hormone.
Eosinophil (Eosinophilia, Eosinophils)
The eosinophils, ordinarily about 2% of the granulocyte count (60 to 75% of the white blood cells), increase in number in the presence of allergic disorders and parasitic infestations.
Exophthalmos
Bulging eyes. See Thyrotoxicosis.
Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).
Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.
Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.
Lymph Nodes (Axillary Nodes, Inguinal Nodes, Lymph Gland, Lymph Glands, Lymph Node, Lymphatic)
Small, bean-shaped nodes at various points throughout the body that function to filter the lymph fluid and attempt to destroy the microorganisms and abnormal cells which collect there. The most common locations are the neck (both sides and front), armpit (axillary) and groin (inguinal), but also under the jaw and behind the ears. Swollen or painful lymph nodes generally result from localized or systemic infection, abscess formation, or malignancy. Other causes of enlarged lymph nodes are extremely rare. Physical examination for lymph nodes includes pressing on them to check for size, texture, warmth, tenderness and mobility. Most lymph nodes can not be felt until they become swollen, and then will only be tender when pressed or massaged. A lymph node that is painful even without touching indicates greater swelling. Lymph nodes can usually be distinguished from other growths because they generally feel small, smooth, round or oval-shaped and somewhat mobile when attempts are made to push them sideways. Because less fat covers the lymph nodes in children, they are easier to feel, even when they are not busy filtering germs or making antibodies. Childrens' nodes enlarge faster, get bigger in response to an infection and stay swollen longer than an adult's. Also known (incorrectly) as lymph glands.
Pituitary (Pituitary Gland)
The pituitary gland is a small (half-inch), bean-shaped organ that hangs down from the lower center of the brain on a stalk attached to another gland, the hypothalamus. Weighing less than one gram, the pituitary gland is often called the "master gland" since it controls the secretion of hormones by other endocrine glands. It regulates many body activities, and is partitioned into front and back lobes. The front lobe is stimulated by the hypothalamus, and produces any one of six different hormones that in turn stimulate the thyroid, adrenal and reproductive glands, and also breast milk production.
Spleen (Splenectomy)
The largest organ of the lymphatic system in the body, located between the stomach and the diaphragm (the muscle below the lungs which provides the motion for breathing). It destroys old red blood cells, produces lymphocytes and plasma cells, and stores blood. It also has other as yet unknown functions. A splenectomy is the surgical removal of the spleen.
Last updated: Sep 28, 2008
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