Current options in the treatment of mast cell mediator-related symptoms in mastocytosis.
Patients with mastocytosis have symptoms related to the tissue response to the release of mediators from mast cells (MC), local mast cell burden or associated non-mast cell hematological disorders. MC contain an array of biologically active mediators in their granules, which are preformed and stored. MC are also able to produce newly generated membrane-derived lipid mediators and are a source of multifunctional cytokines. Mediator-related symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric disturbances; these symptoms are variably controlled by adequate medications. Management of patients within all categories of mastocytosis includes: a) a careful counseling of patients (parents in pediatric cases) and care providers, b) avoidance of factors triggering acute mediator release, c) treatment of acute and chronic MC-mediator symptoms and, if indicated, d) an attempt for cytoreduction and treatment of organ infiltration by mast cells.
Escribano
Luis
L
Unidad de Mastocitosis, Laboratorio K.Frank Austen, Hospital Ramón y Cajal, Red Española de Mastocitosis, Madrid, Spain. lescribano.hrc@salud.madrid.org
Akin
Cem
C
Castells
Mariana
M
Schwartz
Lawrence B
LB
D000818:Animals; D053818:Chymases; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D018836:Inflammation Mediators / Q000502:physiology; D008407:Mast Cells / Q000473:pathology*; D008415:Mastocytosis / Q000188:drug therapy / Q000473:pathology*; D012697:Serine Endopeptidases / Q000494:pharmacology; D053802:Tryptases
D006633:Histamine Antagonists; D018836:Inflammation Mediators; D012697:Serine Endopeptidases; D053818:Chymases; D053802:Tryptases