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Mastocytosis

Mastocytosis - systemeic and diffuse

What is Mastocytosis?

Mastocytosis is a disorder characterized by mast cell proliferation and accumulation within various organs, most commonly the skin.

What are mast cells?

Mast cells are a kind of blood cell made by your bone marrow. They're part of your immune system that helps you fight off infections. There are more of these cells in the skin, the lungs and the intestines than in other parts of your body. Mast cells make a chemical called histamine. Normally, this chemical serves as a kind of alarm to let the immune system know that an infection is attacking part of the body. Histamine can cause swelling, itching and redness when your body reacts to something like an insect bite or a bee sting.

Types of Mastocytosis

There are two types of mastocytosis: cutaneous (skin) and systemic. There are different types of cutaneous and systemic forms. The most common cutaneous form is called urticaria pigmentosa. Urticaria pigmentosa occurs when mast cells get into the skin. Urticaria pigmentosa was first reported in the scientific literature in 1933.

Systemic mastocytosis is caused by the accumulation of mast cells in the tissues. It can affect organs such as the liver, spleen, bone marrow, and small intestine.

Causes of Mastocytosis

Mastocytosis probably is a hyperplastic response to an abnormal stimulus. We don't know why some people have too many mast cells. We do know some things that trigger the release of histamine from mast cells and cause the symptoms of mastocytosis. Symptoms may be triggered by cold or heat, certain medicines, emotional stress and insect bites. The triggers aren't the same in every person.

Systemic mastocytosis is caused when mast cells collect in the tissues and can affect organs such as the liver, spleen, lymph nodes, and bone marrow.

Symptoms of Mastocytosis

Chemicals released by mast cells cause changes in the immune system leading to typical allergy symptoms such as itching, abdominal cramping, and even anaphylaxis (dangerously low blood pressure).

The symptoms are different, depending on where the extra mast cells are. When too many mast cells exist in a person's body, the additional chemicals can cause

  • Bone or muscle pain
  • Abdominal discomfort
  • Nausea and vomiting
  • Stomach ulcers
  • Diarrhea
  • Skin lesions
  • Episodes of very low blood pressure and faintness
  • Shock

Diagnosis of Mastocytosis

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

Treatment of Mastocytosis

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.


 


 
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