Division of Pediatric Hematology, The Johns Hopkins University, Baltimore, MD
21205, USA.
Unregulated activation of mast cells can contribute to the
pathogenesis of inflammatory and allergic diseases, including asthma, rheumatoid
arthritis, inflammatory bowel disease, and multiple sclerosis. Absence of mast
cells in animal models can lead to impairment in the innate immune response to
parasites and bacterial infections. Aberrant clonal accumulation and
proliferation of mast cells can result in a variety of diseases ranging from
benign cutaneous mastocytosis to systemic mastocytosis or mast cell leukemia.
Understanding mast cell differentiation provides important insights into
mechanisms of lineage selection during hematopoiesis and can provide targets for
new drug development to treat mast cell disorders. In this review, we discuss
controversies related to development, sites of origin, and the transcriptional
program of mast cells.
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