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

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:

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

Disclosure statement

Michel Arock receives consultant Fees from Blueprint Medicine, Deciphera and Novartis. Karin Hartmann receives consultant Fees from Allergopharma, ALK, Blueprint, Deciphera, Menarini and Novartis, lectures Fees from Blueprint and Novartis and travel Support from ALK. Karl Sotlar receives speakers Honoraria from Nanostring, Novartis, Pfizer and Beckman-Coulter, consultant Fees from Novartis, Pfizer and Nanostring, and travel Support from Nanostring and Novartis. Peter Valent receives consultant Fees from Novartis, Blueprint and Deciphera. The other authors declare no conflict of interest in relation with the manuscript.

Additional information

Funding

Karin Hartmann receives research support from Euroimmun and Thermofisher. Karl Sotlar receives research support by Novartis. Peter Valent is supported by the Austrian Science Funds (FWF) project F4704-B20. The other authors declare no funding in relation with the manuscript.