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Verfasst von:Fuchs, David [VerfasserIn]   i
 Kilbertus, Alex [VerfasserIn]   i
 Kofler, Karin [VerfasserIn]   i
 von Bubnoff, Nikolas [VerfasserIn]   i
 Shoumariyeh, Khalid [VerfasserIn]   i
 Zanotti, Roberta [VerfasserIn]   i
 Bonadonna, Patrizia [VerfasserIn]   i
 Scaffidi, Luigi [VerfasserIn]   i
 Doubek, Michael [VerfasserIn]   i
 Elberink, Hanneke Oude [VerfasserIn]   i
 Span, Lambert F. R. [VerfasserIn]   i
 Hermine, Olivier [VerfasserIn]   i
 Elena, Chiara [VerfasserIn]   i
 Benvenuti, Pietro [VerfasserIn]   i
 Yavuz, Akif Selim [VerfasserIn]   i
 Brockow, Knut [VerfasserIn]   i
 Zink, Alexander [VerfasserIn]   i
 Aberer, Elisabeth [VerfasserIn]   i
 Gorska, Aleksandra [VerfasserIn]   i
 Romantowski, Jan [VerfasserIn]   i
 Hadzijusufovic, Emir [VerfasserIn]   i
 Fortina, Anna Belloni [VerfasserIn]   i
 Caroppo, Francesca [VerfasserIn]   i
 Perkins, Cecelia [VerfasserIn]   i
 Illerhaus, Anja [VerfasserIn]   i
 Panse, Jens [VerfasserIn]   i
 Vucinic, Vladan [VerfasserIn]   i
 Jawhar, Mohamad [VerfasserIn]   i
 Sabato, Vito [VerfasserIn]   i
 Triggiani, Massimo [VerfasserIn]   i
 Parente, Roberta [VerfasserIn]   i
 Bergström, Anna [VerfasserIn]   i
 Breynaert, Christine [VerfasserIn]   i
 Gotlib, Jason [VerfasserIn]   i
 Reiter, Andreas [VerfasserIn]   i
 Hartmann, Karin [VerfasserIn]   i
 Niedoszytko, Marek [VerfasserIn]   i
 Arock, Michel [VerfasserIn]   i
 Kluin-Nelemans, Hanneke C. [VerfasserIn]   i
 Sperr, Wolfgang R. [VerfasserIn]   i
 Greul, Rosemarie [VerfasserIn]   i
 Valent, Peter [VerfasserIn]   i
Titel:Scoring the risk of having systemic mastocytosis in adult patients with mastocytosis in the skin
Verf.angabe:David Fuchs, Alex Kilbertus, Karin Kofler, Nikolas von Bubnoff, Khalid Shoumariyeh, Roberta Zanotti, Patrizia Bonadonna, Luigi Scaffidi, Michael Doubek, Hanneke Oude Elberink, Lambert F. R. Span, Olivier Hermine, Chiara Elena, Pietro Benvenuti, Akif Selim Yavuz, Knut Brockow, Alexander Zink, Elisabeth Aberer, Aleksandra Gorska, Jan Romantowski, Emir Hadzijusufovic, Anna Belloni Fortina, Francesca Caroppo, Cecelia Perkins, Anja Illerhaus, Jens Panse, Vladan Vucinic, Mohamad Jawhar, Vito Sabato, Massimo Triggiani, Roberta Parente, Anna Bergström, Christine Breynaert, Jason Gotlib, Andreas Reiter, Karin Hartmann, Marek Niedoszytko, Michel Arock, Hanneke C. Kluin-Nelemans, Wolfgang R. Sperr, Rosemarie Greul, Peter Valent
E-Jahr:2021
Jahr:April 2021
Umfang:8 S.
Fussnoten:Gesehen am 14.07.2021
Titel Quelle:Enthalten in: The journal of allergy and clinical immunology / In practice
Ort Quelle:Amsterdam [u.a.] : Elsevier, 2013
Jahr Quelle:2021
Band/Heft Quelle:9(2021), 4, Seite 1705-1712.e4
ISSN Quelle:2213-2201
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.
DOI:doi:10.1016/j.jaip.2020.12.022
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext: https://doi.org/10.1016/j.jaip.2020.12.022
 Volltext: https://www.sciencedirect.com/science/article/pii/S2213219820313544
 DOI: https://doi.org/10.1016/j.jaip.2020.12.022
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:Cutaneous mastocytosis
 Mast cell disease
 Mastocytosis
 Risk score
 Systemic mastocytosis
 Tryptase
K10plus-PPN:1762898101
Verknüpfungen:→ Zeitschrift

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