title: Systemic mastocytosis.
abstract: Systemic mastocytosis (SM) is a clonal disorder of hematopoietic system characterized by abnormal growth and accumulation of mast cells in various tissues. Its clinical spectrum ranges from mild disease to an aggressive course with life-threatening conditions. Some of the clinical signs or symptoms of SM (hyperhidrosis, syncope and hypotensive/tachycardiac attacks) require consideration of pheochromocytoma and carcinoid syndrome in the differential diagnosis. The diagnosis relies on the demonstration of mast cell aggregates in bone marrow or extracutaneous tissues. The World Health Organization categorizes SM into 6 variants: indolent SM, SM with associated clonal hematological nonmast cell lineage disease, aggressive SM, mast cell leukemia, mast cell sarcoma and extracutaneous mastocytosis. Patients with indolent SM have a favorable prognosis with a life expectancy comparable with the healthy population, and symptomatic treatment is usually sufficient. However, more aggressive forms may be life threatening, and cytoreductive treatment is indicated in most cases.
authors.affiliation: Department of Internal Medicine, Hacettepe University Medical School, Ankara, Turkey. sendidem2002@yahoo.com
authors.forename: Didem
authors.initials: D
authors.lastname: Ozdemir
authors.forename: Selcuk
authors.initials: S
authors.lastname: Dagdelen
authors.forename: Tomris
authors.initials: T
authors.lastname: Erbas
mesh_terms: D019070:Cell Lineage; D006801:Humans; D008407:Mast Cells / Q000473:pathology / Q000502:physiology; D034721:Mastocytosis, Systemic / Q000145:classification / Q000175:diagnosis* / Q000473:pathology* / Q000503:physiopathology*; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D053802:Tryptases / Q000097:blood; D014944:World Health Organization
chemical_list: D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases