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

Study Group of the European Competence Network on Mastocytosis (ECNM)

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalJournal of Allergy and Clinical Immunology: In Practice
DOIs
Publication statusAccepted/In press - Jan 1 2018

Fingerprint

Systemic Mastocytosis
Mastocytosis
Mental Competency
Registries
Cutaneous Mastocytosis
Hematologic Neoplasms
Pathology
Survival

Keywords

  • Diagnostic criteria
  • Mastocytosis
  • Prognosis
  • Therapy
  • WHO classification

ASJC Scopus subject areas

  • Immunology and Allergy

Cite this

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

In: Journal of Allergy and Clinical Immunology: In Practice, 01.01.2018.

Research output: Contribution to journalArticle

@article{6fae184dab714951a80ba34d278c6836,
title = "The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives",
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.",
keywords = "Diagnostic criteria, Mastocytosis, Prognosis, Therapy, WHO classification",
author = "{Study Group of the European Competence Network on Mastocytosis (ECNM)} and Peter Valent and {Oude Elberink}, {Joanna N.G.} and Aleksandra Gorska and Magdalena Lange and Roberta Zanotti and {van Anrooij}, Bj{\"o}rn and Massimiliano Bonifacio and Patrizia Bonadonna and Gleixner, {Karoline V.} and Emir Hadzijusufovic and Cecelia Perkins and Karin Hartmann and Anja Illerhaus and Serena Merante and Chiara Elena and Khalid Shoumariyeh and {von Bubnoff}, Nikolas and Roberta Parente and Massimo Triggiani and Juliana Schwaab and Mohamad Jawhar and Francesca Caroppo and Fortina, {Anna Belloni} and Knut Brockow and {David Fuchs}, Fuchs and Rosemarie Greul and Yavuz, {Akif Selim} and Michael Doubek and Mattias Mattsson and Hans Hagglund and Jens Panse and Vito Sabato and Elisabeth Aberer and Al-Ali, {Haifa Kathrin} and Morren, {Marie Anne} and Judit Varkonyi and Alexander Zink and Marek Niedoszytko and Dietger Niederwieser and Luca Malcovati and Andreas Reiter and Vanessa Kennedy and Jason Gotlib and Olivier Lortholary and Olivier Hermine and Michel Arock and Hanneke Kluin-Nelemans and Sperr, {Wolfgang R.}",
year = "2018",
month = "1",
day = "1",
doi = "10.1016/j.jaip.2018.09.024",
language = "English",
journal = "Journal of Allergy and Clinical Immunology: In Practice",
issn = "2213-2198",
publisher = "American Academy of Allergy, Asthma and Immunology",

}

TY - JOUR

T1 - The Data Registry of the European Competence Network on Mastocytosis (ECNM)

T2 - Set Up, Projects, and Perspectives

AU - Study Group of the European Competence Network on Mastocytosis (ECNM)

AU - Valent, Peter

AU - Oude Elberink, Joanna N.G.

AU - Gorska, Aleksandra

AU - Lange, Magdalena

AU - Zanotti, Roberta

AU - van Anrooij, Björn

AU - Bonifacio, Massimiliano

AU - Bonadonna, Patrizia

AU - Gleixner, Karoline V.

AU - Hadzijusufovic, Emir

AU - Perkins, Cecelia

AU - Hartmann, Karin

AU - Illerhaus, Anja

AU - Merante, Serena

AU - Elena, Chiara

AU - Shoumariyeh, Khalid

AU - von Bubnoff, Nikolas

AU - Parente, Roberta

AU - Triggiani, Massimo

AU - Schwaab, Juliana

AU - Jawhar, Mohamad

AU - Caroppo, Francesca

AU - Fortina, Anna Belloni

AU - Brockow, Knut

AU - David Fuchs, Fuchs

AU - Greul, Rosemarie

AU - Yavuz, Akif Selim

AU - Doubek, Michael

AU - Mattsson, Mattias

AU - Hagglund, Hans

AU - Panse, Jens

AU - Sabato, Vito

AU - Aberer, Elisabeth

AU - Al-Ali, Haifa Kathrin

AU - Morren, Marie Anne

AU - Varkonyi, Judit

AU - Zink, Alexander

AU - Niedoszytko, Marek

AU - Niederwieser, Dietger

AU - Malcovati, Luca

AU - Reiter, Andreas

AU - Kennedy, Vanessa

AU - Gotlib, Jason

AU - Lortholary, Olivier

AU - Hermine, Olivier

AU - Arock, Michel

AU - Kluin-Nelemans, Hanneke

AU - Sperr, Wolfgang R.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - Diagnostic criteria

KW - Mastocytosis

KW - Prognosis

KW - Therapy

KW - WHO classification

UR - http://www.scopus.com/inward/record.url?scp=85056241477&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85056241477&partnerID=8YFLogxK

U2 - 10.1016/j.jaip.2018.09.024

DO - 10.1016/j.jaip.2018.09.024

M3 - Article

AN - SCOPUS:85056241477

JO - Journal of Allergy and Clinical Immunology: In Practice

JF - Journal of Allergy and Clinical Immunology: In Practice

SN - 2213-2198

ER -