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Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin
D. Fuchs, A. Kilbertus, K. Kofler, N. von Bubnoff, K. Shoumariyeh, R. Zanotti, P. Bonadonna, L. Scaffidi, M. Doubek, HO. Elberink, LFR. Span, O. Hermine, C. Elena, P. Benvenuti, AS. Yavuz, K. Brockow, A. Zink, E. Aberer, A. Gorska, J....
Language English Country United States
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Adult MeSH
- Bone Marrow MeSH
- Mastocytosis, Cutaneous * diagnosis epidemiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Mastocytosis * MeSH
- Mast Cells MeSH
- Mastocytosis, Systemic * diagnosis epidemiology MeSH
- Tryptases MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
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.
Allergy Unit Verona University Hospital Verona Italy
Department for Hematology and Internal Oncology Kepler University Hospital Linz Austria
Department of Allergology Medical University of Gdańsk Gdańsk Poland
Department of Dermatology and Venereology Medical University of Graz Graz Austria
Department of Dermatology University of Cologne Cologne Germany
Department of Hematology Oncology IRCCS Policlinico San Matteo Foundation Pavia Pavia Italy
Division of Allergy Department of Dermatology University of Basel Basel Switzerland
Division of Hematology Department of Internal Medicine University of Istanbul Istanbul Turkey
German Cancer Consortium Partner site Freiburg Freiburg Germany
Johannes Kepler University Linz Austria
Ludwig Boltzmann Institute for Hematology and Oncology Medical University of Vienna Vienna Austria
Pediatric Dermatology Unit Department of Medicine University of Padua Padua Italy
Section of Hematology Department of Medicine Verona University Hospital Verona Italy
Stanford Cancer Institute Stanford University School of Medicine Stanford Calif
References provided by Crossref.org
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- $a Fuchs, David $u Department for Hematology and Internal Oncology, Kepler University Hospital, Linz, Austria; Johannes Kepler University, Linz, Austria. Electronic address: mail@davidfuchs.at
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- $a Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin / $c D. Fuchs, A. Kilbertus, K. Kofler, N. von Bubnoff, K. Shoumariyeh, R. Zanotti, P. Bonadonna, L. Scaffidi, M. Doubek, HO. Elberink, LFR. Span, O. Hermine, C. Elena, P. Benvenuti, AS. Yavuz, K. Brockow, A. Zink, E. Aberer, A. Gorska, J. Romantowski, E. Hadzijusufovic, AB. Fortina, F. Caroppo, C. Perkins, A. Illerhaus, J. Panse, V. Vucinic, M. Jawhar, V. Sabato, M. Triggiani, R. Parente, A. Bergström, C. Breynaert, J. Gotlib, A. Reiter, K. Hartmann, M. Niedoszytko, M. Arock, HC. Kluin-Nelemans, WR. Sperr, R. Greul, P. Valent
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- $a 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.
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