Fuchs, David, Kilbertus, Alex, Kofler, Karin, von Bubnoff, Nikolas, Shoumariyeh, Khalid, Zanotti, Roberta, Bonadonna, Patrizia, Scaffidi, Luigi, Doubek, Michael, Elberink, Hanneke Oude, Span, Lambert F. R., Hermine, Olivier, Elena, Chiara, Benvenuti, Pietro, Yavuz, Akif Selim, Brockow, Knut, Zink, Alexander ORCID: 0000-0001-9313-6588, Aberer, Elisabeth, Gorska, Aleksandra, Romantowski, Jan ORCID: 0000-0002-0487-0957, Hadzijusufovic, Emir ORCID: 0000-0001-7409-4204, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Caroppo, Francesca ORCID: 0000-0003-3583-0816, Perkins, Cecelia, Illerhaus, Anja, Panse, Jens, Vucinic, Vladan, Jawhar, Mohamad, Sabato, Vito, Triggiani, Massimo, Parente, Roberta, Bergstrom, Anna, Breynaert, Christine ORCID: 0000-0002-7368-1068, Gotlib, Jason, Reiter, Andreas, Hartmann, Karin ORCID: 0000-0002-4595-8226, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Sperr, Wolfgang R., Greul, Rosemarie and Valent, Peter ORCID: 0000-0003-0456-5095 (2021). Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin. J. Allergy Clin. Immunol.-Pract., 9 (4). S. 1705 - 1717. AMSTERDAM: ELSEVIER. ISSN 2213-2201

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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. (C) 2020 American Academy of Allergy, Asthma & Immunology

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kilbertus, AlexUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kofler, KarinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scaffidi, LuigiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elberink, Hanneke OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Span, Lambert F. R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Benvenuti, PietroUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Aberer, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Romantowski, JanUNSPECIFIEDorcid.org/0000-0002-0487-0957UNSPECIFIED
Hadzijusufovic, EmirUNSPECIFIEDorcid.org/0000-0001-7409-4204UNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDorcid.org/0000-0003-3583-0816UNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vucinic, VladanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bergstrom, AnnaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Breynaert, ChristineUNSPECIFIEDorcid.org/0000-0002-7368-1068UNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Greul, RosemarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
URN: urn:nbn:de:hbz:38-607632
DOI: 10.1016/j.jaip.2020.12.022
Journal or Publication Title: J. Allergy Clin. Immunol.-Pract.
Volume: 9
Number: 4
Page Range: S. 1705 - 1717
Date: 2021
Publisher: ELSEVIER
Place of Publication: AMSTERDAM
ISSN: 2213-2201
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
KIT D816V MUTATION; EUROPEAN COMPETENCE NETWORK; MAST-CELL ACTIVATION; BONE-MINERAL DENSITY; I TREAT PATIENTS; QUALITY-OF-LIFE; DIAGNOSTIC ALGORITHM; PERIPHERAL-BLOOD; TRYPTASE LEVELS; CLASSIFICATIONMultiple languages
Allergy; ImmunologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60763

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