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Cardiovascular symptoms in patients with systemic mast cell activation disease

Published:December 27, 2015DOI:https://doi.org/10.1016/j.trsl.2015.12.012
      Traditionally, mast cell activation disease (MCAD) has been considered as just one rare (neoplastic) disease, mastocytosis, focused on the mast cell (MC) mediators tryptase and histamine and the suggestive, blatant symptoms of flushing and anaphylaxis. Recently another form of MCAD, the MC activation syndrome, has been recognized featuring inappropriate MC activation with little to no neoplasia and likely much more heterogeneously clonal and far more prevalent than mastocytosis. Increasing expertise and appreciation has been established for the truly very large menagerie of MC mediators and their complex patterns of release, engendering complex, nebulous presentations of chronic and acute illness best characterized as multisystem polymorbidity of generally inflammatory ± allergic theme. We describe the pathogenesis of MCAD with a particular focus on clinical cardiovascular symptoms and the therapeutic options for MC mediator-induced cardiovascular symptoms.

      Abbreviations:

      H1R (histamine H1 receptor), H2R (histamine H2 receptor), MC (mast cell), MCAD (mast cell activation disease), MCAS (mast cell activation syndrome), MMP (matrix metalloproteinase), NE (norepinephrine), SM (systemic mastocytosis)
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