Scoring the risk of having systemic mastocytosis in adult patients with mastocytosis in the skin

Fuchs, David and Kilbertus, Alex and Kofler, Karin and von Bubnoff, Nikolas and Shoumariyeh, Khalid and Zanotti, Roberta and Bonadonna, Patrizia and Scaffidi, Luigi and Doubek, Michael and Elberink, Hanneke Oude and Span, Lambert F. R. and Hermine, Olivier and Elena, Chiara and Benvenuti, Pietro and Yavuz, Akif Selim and Brockow, Knut and Zink, Alexander and Aberer, Elisabeth and Gorska, Aleksandra and Romantowski, Jan and Hadzijusufovic, Emir and Fortina, Anna Belloni and Caroppo, Francesca and Perkins, Cecelia and Illerhaus, Anja and Panse, Jens and Vucinic, Vladan and Jawhar, Mohamad and Sabato, Vito and Triggiani, Massimo and Parente, Roberta and Bergström, Anna and Breynaert, Christine and Gotlib, Jason and Reiter, Andreas and Hartmann, Karin and Niedoszytko, Marek and Arock, Michel and Kluin-Nelemans, Hanneke C. and Sperr, Wolfgang R. and Greul, Rosemarie and Valent, Peter. (2021) Scoring the risk of having systemic mastocytosis in adult patients with mastocytosis in the skin. The Journal of Allergy and Clinical Immunology: In Practice, 9 (4). pp. 1705-1712.e4.

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Official URL: https://edoc.unibas.ch/80321/

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Abstract

We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 ± 13.3 years. The median serum tryptase level amounted to 29.3 ± 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.

Faculties and Departments:	03 Faculty of Medicine > Bereich Spezialfächer (Klinik) > Dermatologie USB > Allergologie (Hartmann) 03 Faculty of Medicine > Departement Klinische Forschung > Bereich Spezialfächer (Klinik) > Dermatologie USB > Allergologie (Hartmann) 03 Faculty of Medicine > Departement Biomedizin > Department of Biomedicine, University Hospital Basel > Allergy and Immunity (Hartmann)
UniBasel Contributors:	Hartmann, Karin
Item Type:	Article, refereed
Article Subtype:	Research Article
Publisher:	Elsevier
ISSN:	2213-2201
Note:	Publication type according to Uni Basel Research Database: Journal article
Identification Number:	doi: 10.1016/j.jaip.2020.12.022 pmid: 33346151
Last Modified:	14 Apr 2021 09:08
Deposited On:	14 Apr 2021 09:08

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