Description, Causes and Risk Factors:
Infiltration of many organ systems by mast cells with varied clinical manifestations that can include fever, weight loss, flushing, bronchospasm, rhinorrhea, palpitations, dyspnea, diarrhea, gastrointestinal bleeding, and hypotension.
Systemic mastocytosis is a condition caused by the accumulation of mast cells in more than one part of the body. Mast cells contain substances such as histamine that regulate allergic reactions. In systemic mastocytosis, mast cells may build up in the bone marrow, internal organs, or in the skin. This condition is usually diagnosed in adults.
There are several different types of systemic mastocytosis. Indolent systemic mastocytosis develops slowly, while aggressive systemic mastocytosis develops more rapidly. Another form, called systemic mastocytosis with associated hemotologic non-mast cell lineage disease (SM-AHNMD), is characterized by the presence of several types of blood disorders. Mast cell leukemia is a rare form of systemic mastocytosis.
The cause of mastocytosis is not fully known although there is thought to be an association with a genetic mutation known as the c-Kit mutation. A genetic mutation happens when normal instructions carried in certain genes become 'scrambled.' This means some of the body's processes will not work in the normal way.
Systemic mastocytosis is thought to be much rarer, although exactly how rare is uncertain. One estimate is that one in 150,000 people in England have systemic mastocytosis.
Initial signs of this disease may include "spots" that look like freckles on the skin of a person' inner thighs or stomach. These spots are called urticaria pigmentosa and can transform into hives and itch if stroked or irritated, or if the skin is exposed to sudden changes in temperature such as a hot shower.
• Shortness of breath.
• Low blood pressure.
• Hives and swelling.
• Nausea and vomiting.
• Uterine cramps/bleeding.
• Musculoskeletal pain.
Diagnosis can include the following studies and should be conducted by a physician with special knowledge of this disorder:
• Skin and bone marrow biopsies.
• Measurement of mast cell mediators in blood and urine.
• Blood count.
• Liver function studies.
• Genetic tests.
The objective of treatment is to control the effects of mast cell released mediators by avoidance of dietary and environmental triggers as well as the use of various medications. Antihistamines are commonly used. Cromolyn sodium (IntalSM), ketotifen (ZaditorTM) and leukotriene-modifying agents are additional medications which may provide benefit. Epinephrine may be required to treat episodes of low blood pressure. More aggressive forms of systemic mastocytosis may require interferon or chemotherapeutic agents.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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