The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives
| Autor: | Magdalena Lange, Jens Panse, Karoline V. Gleixner, Massimo Triggiani, Elisabeth Aberer, Andreas Reiter, Nikolas von Bubnoff, Roberta Parente, Haifa Kathrin Al-Ali, Knut Brockow, Serena Merante, Olivier Hermine, Akif Selim Yavuz, Patrizia Bonadonna, Chiara Elena, Anja Illerhaus, Olivier Lortholary, Marek Niedoszytko, Hans Hägglund, Bjorn van Anrooij, David Fuchs, Hanneke C. Kluin-Nelemans, Dietger Niederwieser, Emir Hadzijusufovic, Luca Malcovati, Wolfgang R. Sperr, Marie-Anne Morren, Jason Gotlib, Michael Doubek, Mattias Mattsson, Francesca Caroppo, Alexander Zink, Rosemarie Greul, Cecelia Perkins, Vanessa E Kennedy, Massimiliano Bonifacio, Mohamad Jawhar, Joanna N G Oude Elberink, Peter Valent, Judit Várkonyi, Roberta Zanotti, Michel Arock, Anna Belloni Fortina, Khalid Shoumariyeh, Aleksandra Górska, Juliana Schwaab, Karin Hartmann, Vito Sabato |
|---|---|
| Přispěvatelé: | Study Grp European Competence |
| Rok vydání: | 2019 |
| Předmět: |
Risk
Pediatrics medicine.medical_specialty Prognostic variable Diagnostic criteria DISORDERS DIAGNOSTIC-CRITERIA International Cooperation Mast cell activation syndrome Disease World Health Organization CLASSIFICATION 03 medical and health sciences 0302 clinical medicine medicine Humans Immunology and Allergy Registries 030212 general & internal medicine Precision Medicine Systemic mastocytosis Competence (human resources) Mastocytosis Prognosis Therapy WHO classification Information Services MUTATIONS business.industry Cutaneous Mastocytosis Network on KIT SYSTEMIC MASTOCYTOSIS ADULTS Mast cell leukemia medicine.disease DELINEATION Europe 030228 respiratory system MAST-CELLS Human medicine medicine.symptom business CELL ACTIVATION SYNDROMES |
| Zdroj: | The journal of allergy and clinical immunology. In practice |
| ISSN: | 2213-2198 |
| DOI: | 10.1016/j.jaip.2018.09.024 |
| Popis: | Mastocytosis is a unique hematologic neoplasm with complex biology and pathology and a variable clinical course. The disease can essentially be divided into cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In adults, SM is diagnosed in most cases and manifests as either indolent or advanced disease. Patients with advanced SM have an unfavorable prognosis with reduced survival. However, so far, little is known about the prevalence of various categories of SM and about prognostic factors. In an attempt to learn more about the behavior and evolution of various forms of CM and SM, the European Competence Network on Mastocytosis (ECNM) initiated a mastocytosis registry in 2012. In this article, the set up and start phase of this registry are described. Until 2018, more than 3000 patients from 12 countries and 25 centers have been enrolled. In a majority of all patients, robust follow-up data and relevant clinical end points are available. Using this data set, a series of registry projects have been launched, with the aim to validate previously identified diagnostic and prognostic variables and to identify new disease-related and patient-related parameters in various forms of mastocytosis. Moreover, the core data set of the registry will be useful to establish multiparametric scoring systems through which prognostication and individualized management of patients with mastocytosis should improve in the foreseeable future. (C) 2019 American Academy of Allergy, Asthma & Immunology |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|