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Systemic mastocytosis

Mastocytosis is a group of rare disorders of both children and adults caused by the presence of too many mast cells (mastocytes) in a person's body. more...

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Pathophysiology

Mast cells are located in connective tissue, including the skin, the linings of the stomach and intestine, and other sites. They may play an important role in helping defend these tissues from disease. By releasing chemical "alarms" such as histamine, mast cells attract other key players of the immune defense system to areas of the body where they are needed.

Mast cells seem to have other roles as well. Because they gather together around wounds, mast cells may play a part in wound healing. For example, the typical itching you feel around a healing scab may be caused by histamine released by mast cells. Researchers also think mast cells may have a role in the growth of blood vessels (angiogenesis). No one with too few or no mast cells has been found, which indicates to some scientists that we may not be able to survive with too few mast cells.

Mast cells express a cell surface receptor termed c-kit (CD117), which is the receptor for scf (stem cell factor). In laboratory studies, scf appears to be important for the proliferation of mast cells, and inhibiting the tyrosine kinase receptor with imatinib (see below) may reduce the symptoms of mastocytosis.

History

Scientists first described urticaria pigmentosa in 1869. Systemic mastocytosis was first reported by scientists in 1936.

Symptoms

Chemicals released by mast cells cause changes in the immune system leading to typical allergy symptoms such as:

  • itching
  • abdominal cramping
  • and even anaphylaxis (shock from allergic or immune causes)

When too many mast cells exist in a person's body, the additional chemicals can cause:

  • Skin lesions
  • Abdominal discomfort
  • Diarrhea
  • Stomach ulcers
  • Episodes of very low blood pressure (including shock) and faintness
  • bone or muscle pain
  • Nausea and vomiting

Diagnosis

Doctors can diagnose urticaria pigmentosa (cutaneous mastocytosis, see below) by seeing the characteristic lesions which are dark-brown and fixed. A small skin sample (biopsy) may help confirm the diagnosis.

By taking a biopsy from a different organ, such as the bone marrow, the doctor can diagnose systemic mastocytosis. Using special techniques on a bone marrow sample, the doctor looks for an increase in mast cells. Another sign of this disorder is high levels of certain mast-cell chemicals and proteins in a person's blood and sometimes in the urine.

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Mastocytosis
From Gale Encyclopedia of Medicine, 4/6/01 by J. Ricker Polsdorfer

Definition

Mastocytosis is a disease characterized by the presence of too many mast cells in various organs and tissues.

Description

The body has a variety of free-roaming cell populations that function as immunogenic agents. Most immunogenic cells fall into the category of white blood cells, but some remain in tissues and are not found in the blood. Mast cells are such a group.

Mast cells are found primarily in the skin and digestive system, including the liver and spleen, and produce histamine, a chemical most famous for its ability to cause itching. Histamine also causes acid indigestion, diarrhea, flushing, heart pounding, headaches, and can even cause the blood pressure to drop suddenly.

Mastocytosis comes in three forms. Most cases produce symptoms but do not shorten life expectancy. The three forms are:

  • Mastocytoma, a benign skin tumor.
  • Urticaria pigmentosa, small collections of mast cells in the skin that manifest as salmon or brown-colored patches.
  • Systemic mastocytosis, the collection of mast cells in the skin, lymph nodes, liver, spleen, gastrointestinal tract, and bones.

Causes & symptoms

The cause of mastocytosis is unknown. People with systemic mastocytosis have bone and joint pain. Peptic ulcers are frequent because of the increased stomach acid stimulated by histamine. Many patients with systemic mastocytosis also develop urticaria pigmentosa. These skin lesions itch when stroked and may become fluid-filled.

Diagnosis

A biopsy of the skin patches aids diagnosis. An elevated level of histamine in the urine or blood is also indicative of mastocytosis.

Treatment

Mastocytoma usually occurs in childhood and clears-up on its own. Urticaria pigmentosa (present alone without systemic disease) also dramatically clears or improves as adolescence approaches.

Several medications are helpful in relieving symptoms of systemic mastocytosis. Antihistamines and drugs that reduce stomach acid are frequently needed. Headaches respond to migraine treatment. A medicine called cromolyn helps with the bowel symptoms. Several other standard and experimental medications have been used.

Prognosis

Mastocytoma and urticaria pigmentosa rarely if ever, develop into systemic mastocytosis, and both spontaneously improve over time. Systemic mastocytosis is only symptomatically treated. There is no known treatment that decreases the number of mast cells within tissue.

Key Terms

Non-steroidal anti-inflammatory drugs (NSAIDs)
Aspirin, ibuprofen, naproxen, and many others.
Peptic ulcer
Ulcers in the stomach and upper duodenum (first portion of the small intestine) caused by stomach acid and a bacterium called .

Further Reading

For Your Information

    Books

  • Austen, K. Frank. "Diseases of immediate type hypersensitivity." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998, pp.1866-1867.
  • Metcalfe, Dean D. "Mastocytosis." In Cecil Textbook of Medicine, edited by J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders, 1996, pp.1435-1437.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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