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In children, the disease involves mostly the skin (cutaneous mastocytosis;\n                    <jats:styled-content style=\"fixed-case\">CM<\/jats:styled-content>\n                    ), whereas in adults, the disease is usually systemic (systemic mastocytosis;\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    ). Advanced\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    variants with end\u2010organ damage and reduced life expectancy have also been described, but are rare. Clinical signs and symptoms in\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    result from excessive mediator release by\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    s and, in aggressive forms, from organ failure related to\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    infiltration. As a consequence, treatment of indolent\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    aims primarily at the control of symptoms caused by\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    mediator release. By contrast, in advanced\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    , such as aggressive\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    ,\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    leukemia, and\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    sarcoma, intensive (chemo)therapy with or without allogeneic stem cell transplantation has to be considered. In addition, activating mutations in\n                    <jats:italic>\n                      <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>\n                    <\/jats:italic>\n                    (mostly\n                    <jats:italic>\n                      <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>\n                    <\/jats:italic>\n                    D816V in adults) are found in most patients with\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    , so that targeted therapies aimed at blocking mutant\n                    <jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content>\n                    variants or\/and downstream signaling pathways are currently being developed. Other targets, such as specific surface antigens expressed on neoplastic\n                    <jats:styled-content style=\"fixed-case\">MC<\/jats:styled-content>\n                    s, might be considered for the development of future therapies in advanced\n                    <jats:styled-content style=\"fixed-case\">SM<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/ejh.12544","type":"journal-article","created":{"date-parts":[[2015,3,9]],"date-time":"2015-03-09T14:14:54Z","timestamp":1425910494000},"page":"474-490","source":"Crossref","is-referenced-by-count":56,"title":["Current treatment options in patients with mastocytosis: status in 2015 and future perspectives"],"prefix":"10.1111","volume":"94","author":[{"given":"Michel","family":"Arock","sequence":"first","affiliation":[{"name":"Molecular Oncology and Pharmacology LBPA CNRS UMR8113 Ecole Normale Sup\u00e9rieure de Cachan Cachan France"},{"name":"Laboratory of Hematology 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