Zeitschriftenaufsatz | 2019

The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives

Autor:in
Valent, Peter; Elberink, J. N. G. Oude; Górska, Aleksandra; Lange, Magdalena; zanotti, roberta; Anrooij, Bjorn van; Bonifacio, Massimiliano; Bonadonna, P.; Gleixner, Karoline; Hadzijusufovic, Emir; Perkins, Cecelia; Hartmann, Karin; Illerhaus, A.; Merante, Serena; Elena, Chiara; Shoumariyeh, Khalid; von Bubnoff, Nikolas; Parente, Roberta; Triggiani, Massimo; Schwaab, Juliana; Jawhara, Mohamad; Caroppo, Francesca; Belloni Fortina, Anna; Brockow, Knut; Fuchs, David; Greul, Rosemarie; Yavuz, Akif Selim; Doubek, Michael; Mattsson, Mattias; Hagglund, Hans; Panse, Jens; Sabato, Vito; Aberer, Elisabeth; al-ali, haifa; Morren, Marie-Anne; Varkonyi, Judit; Zink, Alexander; Niedoszytko, Marek; Niederwieser, Dietger; Malcovati, Luca; Reiter, Andreas; Kennedy, Vanessa E.; Gotlib, Jason; Lortholary, Olivier; Hermine, Olivier; Arock, Michel; Kluin-Nelemans, Hanneke C.; Sperr, Wolfgang R.
Publikationen als Autor:in / Herausgeber:in der Vetmeduni
Hadzijusufovic, Emir
Journal
Journal Of Allergy And Clinical Immunology-In Practice
Schlagwörter
Mastocytosis; Prognosis; WHO classification; Diagnostic criteria; Therapy
Dokumenten­typ
Übersichtsarbeit
ISSN/eISSN
2213-2198 - 2213-2201
DOI
Öffnen URL 10.1016/j.jaip.2018.09.024
WoS ID
Öffnen URL WOS:000454522500010
PubMed ID
Öffnen URL MEDLINE:30416055

Weitere Details

Band
7
Startseite
81
Letzte Seite
87
Nummer
1
Seiten­anzahl
7
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

Download
 RDF/XML-Format
 JSON-LD-Format
 Turtle-Format
 N3-Format