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Genetic and Rare Diseases Information Center (GARD)

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Systemic mastocytosis


Other Names for this Disease

  • Agressive systemic mastocytosis
  • Indolent systemic mastocytosis
  • Mast cell leukemia
  • SMCD
  • Systemic mast cell disease
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Overview

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. Treatment is generally based on a person's symptoms, but it may include antihistamines (to prevent the effect of mast cell histamine) or chemotherapy for more aggressive forms.[1]

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.[2]
Last updated: 10/5/2012

References

  1. Mastocytosis. National Institute of Allergy and Infectious Diseases. January 2011; http://www.niaid.nih.gov/topics/mastocytosis/Pages/Default.aspx. Accessed 5/15/2013.
  2. All About Mastocytosis. The Mastocytosis Society, Inc. 2011; http://tmsforacure.org/patients/mastocytosis_explained_2.php. Accessed 10/5/2012.
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Basic Information

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Other Names for this Disease
  • Agressive systemic mastocytosis
  • Indolent systemic mastocytosis
  • Mast cell leukemia
  • SMCD
  • Systemic mast cell disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.