Advances in the classification and treatment of mastocytosis: current status and outlook toward the future
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 mastocyt...
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| Main Authors: | , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
March 2, 2017
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| In: |
Cancer research
Year: 2017, Volume: 77, Issue: 6, Pages: 1261-1270 |
| ISSN: | 1538-7445 |
| DOI: | 10.1158/0008-5472.CAN-16-2234 |
| Online Access: | Verlag, Volltext: http://dx.doi.org/10.1158/0008-5472.CAN-16-2234 Verlag, Volltext: http://cancerres.aacrjournals.org/content/77/6/1261 
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| Author Notes: | Peter Valent, Cem Akin, Karin Hartmann, Gunnar Nilsson, Andreas Reiter, Olivier Hermine, Karl Sotlar, Wolfgang R. Sperr, Luis Escribano, Tracy I. George, Hanneke C. Kluin-Nelemans, Celalettin Ustun, Massimo Triggiani, Knut Brockow, Jason Gotlib, Alberto Orfao, Lawrence B. Schwartz, Sigurd Broesby-Olsen, Carsten Bindslev-Jensen, Petri T. Kovanen, Stephen J. Galli, K. Frank Austen, Daniel A. Arber, Hans-Peter Horny, Michel Arock, and Dean D. Metcalfe |
| Summary: | 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. |
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| Item Description: | Published online first March 2, 2017 Gesehen am 24.10.2018 |
| Physical Description: | Online Resource |
| ISSN: | 1538-7445 |
| DOI: | 10.1158/0008-5472.CAN-16-2234 |