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Journal Articles Journal of Allergy and Clinical Immunology: In Practice Year : 2021

Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin

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Alex Kilbertus
  • Function : Author
Karin Kofler
  • Function : Author
Nikolas von Bubnoff
  • Function : Author
Khalid Shoumariyeh
  • Function : Author
Roberta Zanotti
  • Function : Author
Patrizia Bonadonna
  • Function : Author
Luigi Scaffidi
  • Function : Author
Michael Doubek
  • Function : Author
Hanneke Oude Elberink
  • Function : Author
Lambert F.R. Span
  • Function : Author
Chiara Elena
  • Function : Author
Pietro Benvenuti
  • Function : Author
Akif Selim Yavuz
  • Function : Author
Knut Brockow
Alexander Zink
  • Function : Author
Elisabeth Aberer
  • Function : Author
Aleksandra Gorska
  • Function : Author
Jan Romantowski
  • Function : Author
Emir Hadzijusufovic
  • Function : Author
Anna Belloni Fortina
  • Function : Author
Francesca Caroppo
  • Function : Author
Cecelia Perkins
  • Function : Author
Anja Illerhaus
  • Function : Author
Jens Panse
  • Function : Author
Vladan Vucinic
  • Function : Author
Mohamad Jawhar
  • Function : Author
Vito Sabato
  • Function : Author
Massimo Triggiani
  • Function : Author
Roberta Parente
  • Function : Author
Anna Bergström
  • Function : Author
Christine Breynaert
  • Function : Author
Jason Gotlib
Andreas Reiter
  • Function : Author
Karin Hartmann
Marek Niedoszytko
Michel Arock
  • Function : Author
Hanneke Kluin-Nelemans
  • Function : Author
Wolfgang Sperr
  • Function : Author
Rosemarie Greul
  • Function : Author

Abstract

Background: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. Objective: To develop a risk score to predict SM in adults with MIS. Methods: 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. Results: 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. Conclusions: 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.

Dates and versions

hal-03258085 , version 1 (11-06-2021)

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Cite

David Fuchs, Alex Kilbertus, Karin Kofler, Nikolas von Bubnoff, Khalid Shoumariyeh, et al.. Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin. Journal of Allergy and Clinical Immunology: In Practice, 2021, 9 (4), pp.1705-1712.e4. ⟨10.1016/j.jaip.2020.12.022⟩. ⟨hal-03258085⟩
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