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Leuk Lymphoma. 2019 Dec 26:1-9. doi: 10.1080/10428194.2019.1703974. [Epub ahead of print]

New developments in the field of mastocytosis and mast cell activation syndromes: a summary of the Annual Meeting of the European Competence Network on Mastocytosis (ECNM) 2019.

Author information

1
INSERM UMRS1138, Centre de Recherche des Cordeliers, Paris, France.
2
Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.
3
Institute of Pathology, University Hospital, Paracelsus Medical University, Salzburg, Austria.
4
Stanford Cancer Institute, Stanford University School of Medicine, Stanford, CA, USA.
5
Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria.
6
Ludwig Boltzmann Institute for Hematology & Oncology, Medical University of Vienna, Vienna, Austria.
7
Division of Allergy, Department of Dermatology, University Hospital Basel, Basel, Switzerland.
8
Department of Biomedicine, University of Basel, Basel, Switzerland.
9
Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Virginia Commonwealth University, Richmond, VA, USA.
10
Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI, USA.
11
Institute of Pathology, Ludwig-Maximilians University, Munich, Germany.

Abstract

Mastocytosis are a group of hematologic neoplasms characterized by an accumulation of atypical mast cells in one or several organs/tissues, often accompanied by mast cell activation. Whereas in children the disease manifestations are mostly limited to the skin, in adults the disease is usually systemic (systemic mastocytosis; SM) and involves the bone marrow and/or other internal organs. Several variants of SM have been defined. Whereas most patients have indolent SM, some patients have advanced SM, which underlines the complexity of SM. In 2002, a European consortium of clinicians and scientists initiated a multidisciplinary, multi-national cooperative network, termed the 'European Competence Network on Mastocytosis' (ECNM), with the aim to improve diagnosis and therapy of patients with mastocytosis and other mast cell activation disorders. Since then, members of the ECNM have organized Annual Meetings in several European countries. The present article provides a summary of advances in the field presented during the 17th Annual ECNM meeting held in Salzburg in October 2019.

KEYWORDS:

KIT; Mast cell activation; classification; mastocytosis; scoring; tyrosine-kinase inhibitors

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