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Scoring the Risk of...
Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin
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- Fuchs, David (author)
- Kepler Univ Hosp, Dept Hematol & Internal Oncol, Linz, Austria.;Johannes Kepler Univ Linz, Linz, Austria.
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- Kilbertus, Alex (author)
- Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Dermatol & Venerol, Linz, Austria.
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- Kofler, Karin (author)
- Kepler Univ Hosp, Dept Hematol & Internal Oncol, Linz, Austria.
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- von Bubnoff, Nikolas (author)
- Univ Freiburg, Med Ctr, Fac Med, Dept Med 1, Freiburg, Germany.;Univ Schleswig Holstein, Med Ctr, Dept Hematol & Oncol, Campus Lubeck, Lubeck, Germany.
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- Shoumariyeh, Khalid (author)
- Univ Freiburg, Med Ctr, Fac Med, Dept Med 1, Freiburg, Germany.;German Canc Consortium DKTK, Partner Site Freiburg, Freiburg, Germany.
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- Zanotti, Roberta (author)
- Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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- Bonadonna, Patrizia (author)
- Verona Univ Hosp, Allergy Unit, Verona, Italy.
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- Scaffidi, Luigi (author)
- Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
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- Doubek, Michael (author)
- Univ Hosp Brno, Brno, Czech Republic.
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- Elberink, Hanneke Oude (author)
- Univ Groningen, Univ Med Ctr Groningen, Dept Internal Med, Sect Allergy, Groningen, Netherlands.
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- Span, Lambert F. R. (author)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
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- Hermine, Olivier (author)
- Univ Paris 05, Paris Cite, Imagine Inst, Ctr Natl Reference Mastocytoses,Sorbonne, Paris, France.
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- Elena, Chiara (author)
- IRCCS Policlin San Matteo Fdn Pavia, Dept Hematol Oncol, Pavia, Italy.
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- Benvenuti, Pietro (author)
- Univ Pavia, Sch Hematol, Dept Hematol Oncol, Pavia, Italy.;IRCCS Policlin San Matteo Fdn, Pavia, Italy.
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- Yavuz, Akif Selim (author)
- Istanbul Univ, Dept Internal Med, Div Hematol, Istanbul, Turkey.
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- Brockow, Knut (author)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Zink, Alexander (author)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany.
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- Aberer, Elisabeth (author)
- Med Univ Graz, Dept Dermatol & Venereol, Graz, Austria.
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- Gorska, Aleksandra (author)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Romantowski, Jan (author)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Hadzijusufovic, Emir (author)
- Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.;Univ Vet Med, Dept Hosp Compan Anim & Horses, Univ Clin Small Anim, Internal Med Small Anim, Vienna, Austria.
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- Fortina, Anna Belloni (author)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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- Caroppo, Francesca (author)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
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- Perkins, Cecelia (author)
- Stanford Univ, Stanford Canc Inst, Sch Med, Stanford, CA 94305 USA.
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- Illerhaus, Anja (author)
- Univ Cologne, Dept Dermatol, Cologne, Germany.
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- Panse, Jens (author)
- Univ Hosp RWTH Aachen, Med Fac, Dept Oncol Hematol Hemostaseol & Stem Cell Transp, Aachen, Germany.
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- Vucinic, Vladan (author)
- Univ Hosp Leipzig, Leipzig, Germany.
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- Jawhar, Mohamad (author)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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- Sabato, Vito (author)
- Univ Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium.
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- Triggiani, Massimo (author)
- Univ Salerno, Dept Med, Div Allergy & Clin Immunol, Salerno, Italy.
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- Parente, Roberta (author)
- Univ Salerno, Dept Med, Div Allergy & Clin Immunol, Salerno, Italy.
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- Bergström, Anna (author)
- Uppsala universitet,Dermatologi och venereologi
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- Breynaert, Christine (author)
- Univ Hosp Leuven, Dept Gen Internal Med Allergy & Clin Immunol MAST, Leuven, Belgium.;Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.
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- Gotlib, Jason (author)
- Stanford Univ, Stanford Canc Inst, Sch Med, Stanford, CA 94305 USA.
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- Reiter, Andreas (author)
- Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
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- Hartmann, Karin (author)
- Univ Cologne, Dept Dermatol, Cologne, Germany.;Univ Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.
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- Niedoszytko, Marek (author)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
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- Arock, Michel (author)
- Paris Sorbonne Univ, Pitie Salpetriere Hosp, Dept Hematol Biol, Paris, France.
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- Kluin-Nelemans, Hanneke C. (author)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
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- Sperr, Wolfgang R. (author)
- Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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- Greul, Rosemarie (author)
- Kepler Univ Hosp, Dept Hematol & Internal Oncol, Linz, Austria.
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- Valent, Peter (author)
- Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
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Kepler Univ Hosp, Dept Hematol & Internal Oncol, Linz, Austria;Johannes Kepler Univ Linz, Linz, Austria. Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Dermatol & Venerol, Linz, Austria. (creator_code:org_t)
- Elsevier, 2021
- 2021
- English.
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In: Journal of Allergy and Clinical Immunology. - : Elsevier. - 2213-2198 .- 2213-2201. ; 9:4, s. 1705-1712.e4
- Related links:
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https://pure.rug.nl/...
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Subject headings
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- 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
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- Mastocytosis
- Mast cell disease
- Tryptase
- Systemic mastocytosis
- Cutaneous mastocytosis
- Risk score
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Fuchs, David
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Kilbertus, Alex
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Kofler, Karin
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von Bubnoff, Nik ...
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Shoumariyeh, Kha ...
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Zanotti, Roberta
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show more...
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Bonadonna, Patri ...
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Scaffidi, Luigi
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Doubek, Michael
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Elberink, Hannek ...
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Span, Lambert F. ...
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Hermine, Olivier
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Elena, Chiara
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Benvenuti, Pietr ...
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Yavuz, Akif Seli ...
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Brockow, Knut
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Zink, Alexander
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Aberer, Elisabet ...
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Gorska, Aleksand ...
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Romantowski, Jan
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Hadzijusufovic, ...
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Fortina, Anna Be ...
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Caroppo, Frances ...
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Perkins, Cecelia
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Illerhaus, Anja
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Panse, Jens
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Vucinic, Vladan
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Jawhar, Mohamad
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Sabato, Vito
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Triggiani, Massi ...
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Parente, Roberta
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Bergström, Anna
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Breynaert, Chris ...
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Gotlib, Jason
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Reiter, Andreas
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Hartmann, Karin
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Niedoszytko, Mar ...
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Arock, Michel
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Kluin-Nelemans, ...
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Sperr, Wolfgang ...
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Greul, Rosemarie
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Valent, Peter
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
- Articles in the publication
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Journal of Aller ...
- By the university
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Uppsala University