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International progn...
International prognostic scoring system for mastocytosis (IPSM) : a retrospective cohort study
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- Sperr, Wolfgang R. (författare)
- Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, A-1090 Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, A-1090 Vienna, Austria
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- Kundi, Michael (författare)
- Med Univ Vienna, Inst Environm Hlth, Vienna, Austria
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- Alvarez-Twose, Ivan (författare)
- Hosp Virgen Valle, Inst Estudios Mastocitosis Castilla Mancha CLMast, Toledo, Spain
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- van Anrooij, Bjorn (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands;Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands
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- Elberink, Joanna N. G. Oude (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands
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- Gorska, Aleksandra (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland
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- Niedoszytko, Marek (författare)
- Med Univ Gdansk, Dept Allergol, Gdansk, Poland
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- Gleixner, Karoline V. (författare)
- Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, A-1090 Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, A-1090 Vienna, Austria
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- Hadzijusufovic, Emir (författare)
- Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, A-1090 Vienna, Austria;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, A-1090 Vienna, Austria;Univ Vet Med, Dept Univ Clin Compan Anim & Horses, Univ Clin Small Anim, Internal Med Small Anim, Vienna, Austria
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- Zanotti, Roberta (författare)
- Verona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy
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- Bonadonna, Patrizia (författare)
- Verona Univ Hosp, Allergy Unit, Verona, Italy
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- Bonifacio, Massimiliano (författare)
- Verona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy
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- Perkins, Cecelia (författare)
- Stanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA
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- Illerhaus, Anja (författare)
- Univ Cologne, Dept Dermatol, Cologne, Germany
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- Elena, Chiara (författare)
- Univ Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy
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- Merante, Serena (författare)
- Univ Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy
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- Shoumariyeh, Khalid (författare)
- Univ Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany
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- von Bubnoff, Nikolas (författare)
- Univ Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany;Univ Schleswig Holstein, Med Ctr, Dept Hematol & Oncol, Campus Lubeck, Lubeck, Germany
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- Parente, Roberta (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy
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- Jawhar, Mohamad (författare)
- Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany
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- Fortina, Anna Belloni (författare)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy
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- Caroppo, Francesca (författare)
- Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy
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- Brockow, Knut (författare)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy, Munich, Germany
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- Zink, Alexander (författare)
- Tech Univ Munich, Sch Med, Dept Dermatol & Allergy, Munich, Germany
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- Fuchs, David (författare)
- Johannes Kepler Univ Linz, Kepler Univ Hosp, Univ Clin Hematol & Internal Oncol, Linz, Austria
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- Kilbertus, Alex J. (författare)
- Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Dermatol & Venerol, Linz, Austria
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- Yavuz, Akif Selim (författare)
- Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey
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- Doubek, Michael (författare)
- Masaryk Univ, Univ Hosp, Brno, Czech Republic;Masaryk Univ, CEITEC, Brno, Czech Republic
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- Hägglund, Hans (författare)
- Uppsala universitet,Hematologi
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- Panse, Jens (författare)
- Dept Oncol, Haematol, Aachen, Germany
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- Sabato, Vito (författare)
- Univ Antwerp, Dept Immunol Allergol Rheumatol, Fac Med & Hlth Sci, Antwerp, Belgium;Antwerp Univ Hosp, Antwerp, Belgium
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- Bretterklieber, Agnes (författare)
- Univ Hosp Graz, Dept Dermatol & Venereol, Graz, Austria
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- Niederwieser, Dietger (författare)
- Univ Hosp Leipzig, Leipzig, Germany
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- Breynaert, Christine (författare)
- Univ Hosp Leuven, KU Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium;Univ Hosp Leuven, MASTeL, Leuven, Belgium
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- Hartmann, Karin (författare)
- Univ Cologne, Dept Dermatol, Cologne, Germany;Univ Basel, Div Allergy, Dept Dermatol, Basel, Switzerland;Univ Basel, Dept Biomed, Basel, Switzerland
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- Triggiani, Massimo (författare)
- Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy
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- Nedoszytko, Boguslaw (författare)
- Med Univ Gdansk, Dept Dermatol, Gdansk, Poland
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- Reiter, Andreas (författare)
- Heidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany
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- Orfao, Alberto (författare)
- Univ Salamanca, Ctr Invest Canc, CSIC, IBMCC,USAL,CIBERONC, Salamanca, Spain;Univ Salamanca, Dept Med, IBSAL, Salamanca, Spain;Univ Salamanca, Serv Gen Citometria, Salamanca, Spain
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- Hermine, Olivier (författare)
- Univ Paris 05, Imagine Inst, Ctr Natl Reference Mastocytoses, Sorbonne Paris Cite, Paris, France
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- Gotlib, Jason (författare)
- Stanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA
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- Arock, Michel (författare)
- Paris Sorbonne Univ, Pitie Salpetriere Hosp, Dept Hematol Biol, UMR8113, Paris, France
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- Kluin-Nelemans, Hanneke C. (författare)
- Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands
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Valent, Peter (författare)
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(creator_code:org_t)
- ELSEVIER SCI LTD, 2019
- 2019
- Engelska.
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Ingår i: The Lancet Haematology. - : ELSEVIER SCI LTD. - 2352-3026. ; 6:12, s. E638-E649
- Relaterad länk:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- Background The WHO classification separates mastocytosis into distinct variants, but prognostication remains a clinical challenge. The aim of this study was to improve prognostication for patients with mastocytosis. Methods We analysed data of the registry of the European Competence Network on Mastocytosis including 1639 patients (age 17-90 years) diagnosed with mastocytosis according to WHO criteria between Jan 12, 1978, and March 16, 2017. Univariate and multivariate analyses with Cox regression were applied to identify prognostic variables predicting survival outcomes and to establish a prognostic score. We validated this International Prognostic Scoring System in Mastocytosis (IPSM) with data of 462 patients (age 17-79 years) from the Spanish network Red Espanola de Mastocitosis diagnosed between Jan 22, 1998, and Nov 2, 2017. Findings The prognostic value of the WHO classification was confirmed in our study (p<0.0001). For patients with non-advanced mastocytosis (n=1380), we identified age 60 years or older (HR 10.75, 95% CI 5.68-20.32) and a concentration of alkaline phosphatase 100 U/L or higher (2.91, 1.60-5.30) as additional independent prognostic variables for overall survival. The resulting scoring system divided patients with non-advanced mastocytosis into three groups: low (no risk factors), intermediate 1 (one risk factor), and intermediate 2 (two risk factors). Overall survival and progression-free survival differed significantly among these groups (p<0.0001). In patients with advanced mastocytosis (n=259), age 60 years or older (HR 2.14, 95% CI 1.42-3.22), a concentration of tryptase 125 ng/mL or higher (1.81, 1.20-2.75), a leukocyte count of 16 x 10(9) per L or higher (1.88, 1.27-2.79), haemoglobin of 11 g/dL or lower (1.71, 1.13-2.57), a platelet count of 100 x 10(9) per L or lower (1.63, 1.13-2.34), and skin involvement (0.46, 0.30-0.69) were prognostic variables. Based on these variables, a separate score for advanced mastocytosis with four risk categories was established, with significantly different outcomes for overall survival and progression-free survival (p<0.0001). The prognostic value of both scores was confirmed in 413 patients with non-advanced disease and 49 with advanced mastocytosis from the validation cohort. Interpretation The IPSM scores for patients with non-advanced and advanced mastocytosis can be used to predict survival outcomes and guide treatment decisions. However, the predictive value of the IPSM needs to be confirmed in forthcoming trials. Copyright (C) 2019 Elsevier Ltd. All rights reserved.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
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Sperr, Wolfgang ...
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Kundi, Michael
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Alvarez-Twose, I ...
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van Anrooij, Bjo ...
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Elberink, Joanna ...
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Gorska, Aleksand ...
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Niedoszytko, Mar ...
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Gleixner, Karoli ...
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Hadzijusufovic, ...
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Zanotti, Roberta
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Bonadonna, Patri ...
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Bonifacio, Massi ...
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Perkins, Cecelia
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Illerhaus, Anja
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Elena, Chiara
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Merante, Serena
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Shoumariyeh, Kha ...
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von Bubnoff, Nik ...
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Parente, Roberta
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Jawhar, Mohamad
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Fortina, Anna Be ...
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Caroppo, Frances ...
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Brockow, Knut
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Zink, Alexander
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Fuchs, David
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Kilbertus, Alex ...
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Yavuz, Akif Seli ...
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Doubek, Michael
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Hägglund, Hans
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Panse, Jens
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Sabato, Vito
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Bretterklieber, ...
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Niederwieser, Di ...
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Breynaert, Chris ...
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Hartmann, Karin
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Triggiani, Massi ...
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Nedoszytko, Bogu ...
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Reiter, Andreas
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Orfao, Alberto
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Hermine, Olivier
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Gotlib, Jason
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Arock, Michel
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Kluin-Nelemans, ...
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Valent, Peter
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