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The condition is based on clonal mast cell proliferation, usually in the skin or bone marrow and only rarely in the gastrointestinal tract or other tissues. In general, mastocytosis has a good prognosis in terms of life expectancy. Rare variants \u2013 including mast cell leukemia, aggressive mastocytosis, and the exceedingly rare mast cell sarcoma \u2013 require cytoreductive therapy. In cases associated with hematological neoplasms, the prognosis depends on the underlying hematologic disorder.<\/jats:p>","DOI":"10.1111\/ddg.13418","type":"journal-article","created":{"date-parts":[[2018,1,13]],"date-time":"2018-01-13T03:50:33Z","timestamp":1515815433000},"page":"42-57","source":"Crossref","is-referenced-by-count":4,"title":["Mastocytosis \u2013 pathogenesis, clinical manifestation and treatment"],"prefix":"10.1111","volume":"16","author":[{"given":"Nicola","family":"Wagner","sequence":"first","affiliation":[{"name":"Department of Dermatology University Medical Center  Erlangen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Staubach","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center  Mainz 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