Mastocytosis is a rare stem cell disorder characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Clinical heterogeneity is a hallmark of mastocytosis. Recent observations of activating mutations in c-kit may help to understand the abnormal growth of mast cells in mastocytosis. However, this mutation alone does not explain the entire clinical heterogeneity of the disease. Reticulin fibrosis is also commonly associated with systemic mastocytosis. Mast cells are known to be the source of fibrogenic cytokines, including platelet-derived growth factor, transforming growth factor-β (TGFβ) and basic fibroblast growth factor (bFGF). Immunohistochemical studies show a close correlation between the mast cell expression of bFGF and the reticulin fibrosis of mastocytosis lesions. The study of cytokine receptor expression also demonstrates that the TGFβ receptor I (RI)-negative cases of mastocytosis are prognostically less favorable than the TGFβRI-positive cases. This finding may be related to the fact that the TGFβR complex functions as a tumor suppressor gene in neoplastic cells.

Keywords:
Mastocytosis, Fibrosis, Cytokine, Cytokine receptor, Basic fibroblast growth factor, Transforming growth factor-β, Transforming growth factor-β receptor
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© 2002 S. Karger AG, Basel
2002
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