Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis
Luis Escribano, Cem Akin, Mariana Castells and Lawrence B. Schwartz
Affiliation: Hospital Ramon y Cajal, Servicio de Hematologia, Carretera de Colmenar Km. 9.1, Madrid 28034, Spain;
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.
Keywords: Mastocytosis, mast cell-mediator related symptoms, treatment
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