Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future
Year: 2017
Type: article
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.
Source: Cancer Research
Authors: 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, Dean D. Metcalfe
Institutions: Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Brigham and Women's Hospital, Harvard University, University of Lübeck, Karolinska University Hospital, Karolinska Institutet, Heidelberg University, University Medical Centre Mannheim, Université Paris Cité, Sorbonne Paris Cité, Université Paris Descartes, Paracelsus Medical University, Universidad de Salamanca, Instituto de Investigación Biomédica de Salamanca, Centro de Investigación del Cáncer, University of New Mexico, University Medical Center Groningen, University of Groningen, University of Minnesota, University of Salerno, Technical University of Munich, Stanford Medicine, Stanford Cancer Institute, Stanford University, Virginia Commonwealth University, Odense University Hospital, Wihuri Research Institute, University of Chicago, Pathologisches Institut, Ludwig-Maximilians-Universität München, École Normale Supérieure Paris-Saclay, Centre National de la Recherche Scientifique, Laboratoire de Biologie et Pharmacologie Appliquée, National Institutes of Health, National Institute of Allergy and Infectious Diseases
Language: English
Topic: Mast cells and histamine
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: green
OpenAlex ID: W2593680283