title: Critical care management of systemic mastocytosis: when every wasp is a killer bee.
abstract: Since the critical care physician will most likely be involved in a life-threatening expression of systemic mastocytosis, recognition of this disease is of utmost importance in the critical care management of these patients. Mastocytosis is a severely under-recognized disease because it typically occurs secondary to another condition and thus may occur more frequently than assumed. In this article, we will review the current knowledge on the treatment of mastocytosis crises with an emphasis on critical care management. Mastocytosis is characterized by the clonal proliferation and accumulation of mast cells in different tissues. Mast cell mediators contain a wide range of biologically active substances that may lead to itching and hives but may ultimately lead to anaphylactic shock caused by the release of histamine and other mediators from mast cells. The mainstay of therapy is the avoidance of potential triggers of mast cell degranulation and, if unsuccessful, blocking the cascade of mast cell mediators. The critical care physician should be well aware of the special precautions which should be kept in mind throughout the management of a mastocytosis crisis to avoid massive mast cell degranulation. Histamine-releasing drugs and certain physical triggers like temperature change should be avoided.
authors.affiliation: Department of Critical Care, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands. w.m.van.den.bergh@umcg.nl.
authors.forename: Hinke Y
authors.initials: HY
authors.lastname: van der Weide
authors.affiliation: Department of Intensive Care, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands. w.m.van.den.bergh@umcg.nl.
authors.forename: David J
authors.initials: DJ
authors.lastname: van Westerloo
authors.affiliation: Department of Critical Care, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands. w.m.van.den.bergh@umcg.nl.
authors.forename: Walter M
authors.initials: WM
authors.lastname: van den Bergh
mesh_terms: D000707:Anaphylaxis / Q000209:etiology / Q000517:prevention & control; D001993:Bronchodilator Agents / Q000627:therapeutic use; D015550:Cell Degranulation; D003422:Critical Care; D004837:Epinephrine / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D015994:Incidence; D008407:Mast Cells / Q000502:physiology; D034721:Mastocytosis, Systemic / Q000175:diagnosis* / Q000628:therapy*; D015995:Prevalence; D012307:Risk Factors; D012769:Shock / Q000209:etiology / Q000628:therapy; D053802:Tryptases / Q000097:blood
chemical_list: D001993:Bronchodilator Agents; D053802:Tryptases; D004837:Epinephrine