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Cancer Res. 2017 Mar 15;77(6):1261-1270. doi: 10.1158/0008-5472.CAN-16-2234. Epub 2017 Mar 2.

Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future.

Author information

1
Department of Medicine I, Division of Hematology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria. peter.valent@meduniwien.ac.at.
2
Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
3
Department of Dermatology, University of Luebeck, Luebeck, Germany.
4
Department of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden.
5
Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
6
Imagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre National de Référence des Mastocytoses, Paris, France.
7
Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria.
8
Department of Medicine I, Division of Hematology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
9
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.
10
Department of Pathology, University of New Mexico, Albuquerque, New Mexico.
11
Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
12
Division of Hematology-Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, Minnesota.
13
Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
14
Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
15
Division of Hematology, Stanford Cancer Institute, Stanford University School of Medicine, Stanford, California.
16
Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Virginia Commonwealth University, Richmond, Virginia.
17
Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
18
Wihuri Research Institute, Helsinki, Finland.
19
Institute of Pathology, University of Chicago, Chicago, Illinois.
20
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
21
LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.
22
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, Maryland.

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.

PMID:
28254862
PMCID:
PMC5354959
DOI:
10.1158/0008-5472.CAN-16-2234
[Indexed for MEDLINE]
Free PMC Article

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