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. 2019 Jan;7(1):81-87.
doi: 10.1016/j.jaip.2018.09.024. Epub 2018 Nov 8.

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

Peter Valent  1 , Joanna N G Oude Elberink  2 , Aleksandra Gorska  3 , Magdalena Lange  4 , Roberta Zanotti  5 , Björn van Anrooij  2 , Massimiliano Bonifacio  5 , Patrizia Bonadonna  6 , Karoline V Gleixner  7 , Emir Hadzijusufovic  8 , Cecelia Perkins  9 , Karin Hartmann  10 , Anja Illerhaus  11 , Serena Merante  12 , Chiara Elena  12 , Khalid Shoumariyeh  13 , Nikolas von Bubnoff  14 , Roberta Parente  15 , Massimo Triggiani  15 , Juliana Schwaab  16 , Mohamad Jawhar  16 , Francesca Caroppo  17 , Anna Belloni Fortina  17 , Knut Brockow  18 , David Fuchs  19 , Rosemarie Greul  19 , Akif Selim Yavuz  20 , Michael Doubek  21 , Mattias Mattsson  22 , Hans Hagglund  23 , Jens Panse  24 , Vito Sabato  25 , Elisabeth Aberer  26 , Haifa Kathrin Al-Ali  27 , Marie-Anne Morren  28 , Judit Varkonyi  29 , Alexander Zink  18 , Marek Niedoszytko  3 , Dietger Niederwieser  27 , Luca Malcovati  12 , Andreas Reiter  16 , Vanessa Kennedy  9 , Jason Gotlib  9 , Olivier Lortholary  30 , Olivier Hermine  31 , Michel Arock  32 , Hanneke Kluin-Nelemans  33 , Wolfgang R Sperr  7 ; Study Group of the European Competence Network on Mastocytosis (ECNM)
Affiliations

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

Peter Valent et al. J Allergy Clin Immunol Pract. 2019 Jan.

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.

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Conflict of interest statement

Conflicts of interest: The authors declare that they have no relevant conflict of interest.

Figures

Figure 1
Figure 1
Recruitment of patients into the ECNM registry over time. The figure shows the number of patients with mastocytosis recruited into the ECNM registry per year. Until June 2018, more than 3000 patients have been enrolled.
Figure 2
Figure 2
Distribution of mastocytosis variants in the total patient cohort. The diagram shows the relative number of patients (N = 2985) in each diagnostic subgroup (variants of mastocytosis) collected in the ECNM registry. MCS, Mast cell sarcoma; MIS, mastocytosis in the skin; SM-AHN, SM with associated hematologic neoplasm; SSM, smoldering SM. MIS is diagnosed in adult patients with skin involvement in whom no BM investigation was performed and who may finally turn out to have SM or CM, based on a later BM biopsy. No children are included in the MIS group.
Figure 3
Figure 3
Survival of patients with SM. A total of 1358 patients with SM with robust follow-up data were examined. Patients were split into SM cases without any B- or C-Findings (blue graph; n = 932), 1 or more B-Finding but no C-Findings (green graph; n = 206), and 1 or more C-Finding (red graph; n = 220). Patients with B- or C-Findings were often found to suffer from an associated hematologic neoplasm (SM-AHN). The survival estimates were established using the method of Kaplan and Meier. Survival was calculated from the date of diagnosis. Differences in survival were found to be statistically significant (P < .05).
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References

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