title: Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future.
abstract: Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.Cancer Res; 77(6); 1261-70. ©2017 AACR.
authors.affiliation: Department of Medicine I, Division of Hematology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria. peter.valent@meduniwien.ac.at.
authors.forename: Peter
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authors.lastname: Valent
authors.affiliation: Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Cem
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authors.lastname: Akin
authors.affiliation: Department of Dermatology, University of Luebeck, Luebeck, Germany.
authors.forename: Karin
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authors.lastname: Hartmann
authors.affiliation: Department of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Gunnar
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authors.lastname: Nilsson
authors.affiliation: Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiter
authors.affiliation: Imagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre National de Référence des Mastocytoses, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.affiliation: Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria.
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.affiliation: Department of Medicine I, Division of Hematology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
authors.forename: Wolfgang R
authors.initials: WR
authors.lastname: Sperr
authors.affiliation: Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Salamanca, Spain.
authors.forename: Luis
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authors.lastname: Escribano
authors.affiliation: Department of Pathology, University of New Mexico, Albuquerque, New Mexico.
authors.forename: Tracy I
authors.initials: TI
authors.lastname: George
authors.affiliation: Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
authors.forename: Hanneke C
authors.initials: HC
authors.lastname: Kluin-Nelemans
authors.affiliation: Division of Hematology-Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, Minnesota.
authors.forename: Celalettin
authors.initials: C
authors.lastname: Ustun
authors.affiliation: Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
authors.forename: Massimo
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authors.lastname: Triggiani
authors.affiliation: Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
authors.forename: Knut
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authors.lastname: Brockow
authors.affiliation: Division of Hematology, Stanford Cancer Institute, Stanford University School of Medicine, Stanford, California.
authors.forename: Jason
authors.initials: J
authors.lastname: Gotlib
authors.affiliation: Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Salamanca, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Virginia Commonwealth University, Richmond, Virginia.
authors.forename: Lawrence B
authors.initials: LB
authors.lastname: Schwartz
authors.affiliation: Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
authors.forename: Sigurd
authors.initials: S
authors.lastname: Broesby-Olsen
authors.affiliation: Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
authors.forename: Carsten
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authors.lastname: Bindslev-Jensen
authors.affiliation: Wihuri Research Institute, Helsinki, Finland.
authors.forename: Petri T
authors.initials: PT
authors.lastname: Kovanen
authors.affiliation: Institute of Pathology, University of Chicago, Chicago, Illinois.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Galli
authors.affiliation: Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: K Frank
authors.initials: KF
authors.lastname: Austen
authors.affiliation: Institute of Pathology, University of Chicago, Chicago, Illinois.
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Arber
authors.affiliation: Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Horny
authors.affiliation: LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Arock
authors.affiliation: Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, Maryland.
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authors.initials: DD
authors.lastname: Metcalfe
mesh_terms: D018450:Disease Progression; D006801:Humans; D008415:Mastocytosis / Q000145:classification* / Q000628:therapy*