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Systemic Mastocytosis
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Systemic mastocytosis (SM) is a disease characterized by an abnormal proliferation and accumulation of a clonal population of mast cells in the tissues including the bone marrow, gastrointestinal tract, and skin. In the most common form, indolent mastocytosis, the symptoms are most often due to the consequences of mast cell activation and include flushing, abdominal pain, and diarrhea. The diagnosis is made using a defined set of criteria based on a tissue biopsy, with special staining and a blood test for tryptase. Treatment is directed at the mast cell activation and specific symptoms such as nausea and abdominal bloating. Differential diagnosis includes the non-clonal form of mast cell activation syndrome and IgE-mediated allergy.
Keywords: mast cell, mast cell activation, mastocytosis, tryptase, flushing, urticaria pigmentosa, dermatographism
This review contains 5 highly rendered figures, 4 tables, and 35 references.
Title: Systemic Mastocytosis
Description:
Systemic mastocytosis (SM) is a disease characterized by an abnormal proliferation and accumulation of a clonal population of mast cells in the tissues including the bone marrow, gastrointestinal tract, and skin.
In the most common form, indolent mastocytosis, the symptoms are most often due to the consequences of mast cell activation and include flushing, abdominal pain, and diarrhea.
The diagnosis is made using a defined set of criteria based on a tissue biopsy, with special staining and a blood test for tryptase.
Treatment is directed at the mast cell activation and specific symptoms such as nausea and abdominal bloating.
Differential diagnosis includes the non-clonal form of mast cell activation syndrome and IgE-mediated allergy.
Keywords: mast cell, mast cell activation, mastocytosis, tryptase, flushing, urticaria pigmentosa, dermatographism
This review contains 5 highly rendered figures, 4 tables, and 35 references.
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