title: Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts.
abstract: Over the past few years, substantial advances have been made in understanding the pathogenesis, evolution, and complexity of mast cell neoplasms. New diagnostic and prognostic parameters and novel therapeutic targets with demonstrable clinical impact have been identified. Several of these new markers, molecular targets, and therapeutic approaches have been validated and translated into clinical practice. At the same time, the classification of mastocytosis and related diagnostic criteria have been refined and updated by the consensus group and the World Health Organization (WHO). As a result, more specific therapies tailored toward prognostic subgroups of patients have been developed. Emerging treatment concepts use drugs directed against KIT and other relevant targets in neoplastic mast cells and will hopefully receive recognition by health authorities in the near future. This article provides an overview of recent developments in the field, with emphasis on the updated WHO classification, refined criteria, additional prognostic parameters, and novel therapeutic approaches. Based on these emerging concepts, the prognosis, quality of life, and survival of patients with advanced mastocytosis are expected to improve in the coming years.
authors.affiliation: Division of Hematology & Hemostaseology and Ludwig Boltzmann Cluster Oncology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: Peter
authors.identifier: 0000-0003-0456-5095
authors.initials: P
authors.lastname: Valent
authors.affiliation: Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; and.
authors.forename: Cem
authors.identifier: 0000-0001-6301-4520
authors.initials: C
authors.lastname: Akin
authors.affiliation: Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.
authors.forename: Dean D
authors.identifier: 0000-0002-7781-8260
authors.initials: DD
authors.lastname: Metcalfe
mesh_terms: D006801:Humans; D008415:Mastocytosis / Q000145:classification* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D058990:Molecular Targeted Therapy / Q000379:methods; D011379:Prognosis; D011788:Quality of Life; D014944:World Health Organization