Valent, Peter ORCID: 0000-0003-0456-5095, Elberink, Joanna N. G. Oude, Gorska, Aleksandra ORCID: 0000-0001-6682-1555, Lange, Magdalena ORCID: 0000-0002-6967-1393, Zanotti, Roberta, van Anrooij, Bjorn, Bonifacio, Massimiliano ORCID: 0000-0003-0716-1686, Bonadonna, Patrizia, Gleixner, Karoline V., Hadzijusufovic, Emir ORCID: 0000-0001-7409-4204, Perkins, Cecelia, Hartmann, Karin ORCID: 0000-0002-4595-8226, Illerhaus, Anja, Merante, Serena, Elena, Chiara, Shoumariyeh, Khalid ORCID: 0000-0001-7969-1972, von Bubnoff, Nikolas, Parente, Roberta ORCID: 0000-0002-4187-6477, Triggiani, Massimo ORCID: 0000-0001-7318-2093, Schwaab, Juliana, Jawhar, Mohamad, Caroppo, Francesca, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Brockow, Knut, Fuchs, David, Greul, Rosemarie, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias ORCID: 0000-0002-9510-8801, Hagglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Al-Ali, Haifa Kathrin, Morren, Marie-Anne, Varkonyi, Judit, Zink, Alexander ORCID: 0000-0001-9313-6588, Niedoszytko, Marek ORCID: 0000-0003-1089-1911, Niederwieser, Dietger, Malcovati, Luca, Reiter, Andreas, Kennedy, Vanessa, Gotlib, Jason, Lortholary, Olivier, Hermine, Olivier, Arock, Michel, Kluin-Nelemans, Hanneke and Sperr, Wolfgang R. (2019). The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives. J. Allergy Clin. Immunol.-Pract., 7 (1). S. 81 - 88. AMSTERDAM: ELSEVIER SCIENCE BV. ISSN 2213-2201

Full text not available from this repository.

Abstract

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

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
Elberink, Joanna N. G. OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDorcid.org/0000-0001-6682-1555UNSPECIFIED
Lange, MagdalenaUNSPECIFIEDorcid.org/0000-0002-6967-1393UNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjornUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonifacio, MassimilianoUNSPECIFIEDorcid.org/0000-0003-0716-1686UNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gleixner, Karoline V.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hadzijusufovic, EmirUNSPECIFIEDorcid.org/0000-0001-7409-4204UNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Merante, SerenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDorcid.org/0000-0001-7969-1972UNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDorcid.org/0000-0002-4187-6477UNSPECIFIED
Triggiani, MassimoUNSPECIFIEDorcid.org/0000-0001-7318-2093UNSPECIFIED
Schwaab, JulianaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Greul, RosemarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mattsson, MattiasUNSPECIFIEDorcid.org/0000-0002-9510-8801UNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aberer, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Al-Ali, Haifa KathrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Morren, Marie-AnneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Varkonyi, JuditUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDorcid.org/0000-0003-1089-1911UNSPECIFIED
Niederwieser, DietgerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Malcovati, LucaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kennedy, VanessaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lortholary, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kluin-Nelemans, HannekeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-140590
DOI: 10.1016/j.jaip.2018.09.024
Journal or Publication Title: J. Allergy Clin. Immunol.-Pract.
Volume: 7
Number: 1
Page Range: S. 81 - 88
Date: 2019
Publisher: ELSEVIER SCIENCE BV
Place of Publication: AMSTERDAM
ISSN: 2213-2201
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
CELL ACTIVATION SYNDROMES; SYSTEMIC MASTOCYTOSIS; MAST-CELLS; DIAGNOSTIC-CRITERIA; CLASSIFICATION; DISORDERS; MUTATIONS; ADULTS; KIT; DELINEATIONMultiple languages
Allergy; ImmunologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/14059

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item