Pathogenesis and Pathology of Mastocytosis

Annu Rev Pathol. 2017 Jan 24:12:487-514. doi: 10.1146/annurev-pathol-052016-100312.

Abstract

Systemic mastocytosis is a clonal disorder of mast cells that may variably present with characteristic skin lesions, episodes of mast cell mediator release, and disturbances of hematopoiesis. No curative therapy presently exists. Conventional management has relied on agents that antagonize mediators released by mast cells, inhibit mediator secretion, or modulate mast cell proliferation. Recent advances in the molecular understanding of the pathophysiology of systemic mastocytosis have provided new therapeutic considerations, including new and novel tyrosine kinase inhibitors.

Keywords: KIT; mast cell mediators; mast cells; mastocytosis.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Mast Cells / pathology*
  • Mastocytosis / pathology*