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  • Sperr, Wolfgang R.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 (författare)

International prognostic scoring system for mastocytosis (IPSM) : a retrospective cohort study

  • Artikel/kapitelEngelska2019

Förlag, utgivningsår, omfång ...

  • ELSEVIER SCI LTD,2019
  • printrdacarrier

Nummerbeteckningar

  • LIBRIS-ID:oai:DiVA.org:uu-400057
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-400057URI
  • https://doi.org/10.1016/S2352-3026(19)30166-8DOI

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  • Språk:engelska
  • Sammanfattning på:engelska

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  • Ämneskategori:ref swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • 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.

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Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Kundi, MichaelMed Univ Vienna, Inst Environm Hlth, Vienna, Austria (författare)
  • Alvarez-Twose, IvanHosp Virgen Valle, Inst Estudios Mastocitosis Castilla Mancha CLMast, Toledo, Spain (författare)
  • van Anrooij, BjornUniv Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands;Univ Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands (författare)
  • Elberink, Joanna N. G. OudeUniv Groningen, Univ Med Ctr Groningen, Dept Allergol, Groningen, Netherlands (författare)
  • Gorska, AleksandraMed Univ Gdansk, Dept Allergol, Gdansk, Poland (författare)
  • Niedoszytko, MarekMed Univ Gdansk, Dept Allergol, Gdansk, Poland (författare)
  • Gleixner, Karoline V.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 (författare)
  • Hadzijusufovic, EmirMed 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 (författare)
  • Zanotti, RobertaVerona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy (författare)
  • Bonadonna, PatriziaVerona Univ Hosp, Allergy Unit, Verona, Italy (författare)
  • Bonifacio, MassimilianoVerona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy (författare)
  • Perkins, CeceliaStanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA (författare)
  • Illerhaus, AnjaUniv Cologne, Dept Dermatol, Cologne, Germany (författare)
  • Elena, ChiaraUniv Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy (författare)
  • Merante, SerenaUniv Pavia, Dept Mol Med, Pavia, Italy;Univ Pavia, Dept Hematol Oncol, Pavia, Italy;Fdn IRCCS Policlin San Matteo, Pavia, Italy (författare)
  • Shoumariyeh, KhalidUniv Freiburg, Fac Med, Med Ctr, Dept Hematol Oncol & Stem Cell Transplantat, Freiburg, Germany (författare)
  • von Bubnoff, NikolasUniv 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 (författare)
  • Parente, RobertaUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy (författare)
  • Jawhar, MohamadHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany (författare)
  • Fortina, Anna BelloniUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy (författare)
  • Caroppo, FrancescaUniv Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy (författare)
  • Brockow, KnutTech Univ Munich, Sch Med, Dept Dermatol & Allergy, Munich, Germany (författare)
  • Zink, AlexanderTech Univ Munich, Sch Med, Dept Dermatol & Allergy, Munich, Germany (författare)
  • Fuchs, DavidJohannes Kepler Univ Linz, Kepler Univ Hosp, Univ Clin Hematol & Internal Oncol, Linz, Austria (författare)
  • Kilbertus, Alex J.Johannes Kepler Univ Linz, Kepler Univ Hosp, Dept Dermatol & Venerol, Linz, Austria (författare)
  • Yavuz, Akif SelimIstanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkey (författare)
  • Doubek, MichaelMasaryk Univ, Univ Hosp, Brno, Czech Republic;Masaryk Univ, CEITEC, Brno, Czech Republic (författare)
  • Hägglund, HansUppsala universitet,Hematologi(Swepub:uu)hanha689 (författare)
  • Panse, JensDept Oncol, Haematol, Aachen, Germany (författare)
  • Sabato, VitoUniv Antwerp, Dept Immunol Allergol Rheumatol, Fac Med & Hlth Sci, Antwerp, Belgium;Antwerp Univ Hosp, Antwerp, Belgium (författare)
  • Bretterklieber, AgnesUniv Hosp Graz, Dept Dermatol & Venereol, Graz, Austria (författare)
  • Niederwieser, DietgerUniv Hosp Leipzig, Leipzig, Germany (författare)
  • Breynaert, ChristineUniv Hosp Leuven, KU Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium;Univ Hosp Leuven, MASTeL, Leuven, Belgium (författare)
  • Hartmann, KarinUniv Cologne, Dept Dermatol, Cologne, Germany;Univ Basel, Div Allergy, Dept Dermatol, Basel, Switzerland;Univ Basel, Dept Biomed, Basel, Switzerland (författare)
  • Triggiani, MassimoUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy (författare)
  • Nedoszytko, BoguslawMed Univ Gdansk, Dept Dermatol, Gdansk, Poland (författare)
  • Reiter, AndreasHeidelberg Univ, Univ Med Mannheim, Med Klin 3, Mannheim, Germany (författare)
  • Orfao, AlbertoUniv Salamanca, Ctr Invest Canc, CSIC, IBMCC,USAL,CIBERONC, Salamanca, Spain;Univ Salamanca, Dept Med, IBSAL, Salamanca, Spain;Univ Salamanca, Serv Gen Citometria, Salamanca, Spain (författare)
  • Hermine, OlivierUniv Paris 05, Imagine Inst, Ctr Natl Reference Mastocytoses, Sorbonne Paris Cite, Paris, France (författare)
  • Gotlib, JasonStanford Univ, Sch Med, Dept Med, Div Hematol, Stanford, CA 94305 USA (författare)
  • Arock, MichelParis Sorbonne Univ, Pitie Salpetriere Hosp, Dept Hematol Biol, UMR8113, Paris, France (författare)
  • Kluin-Nelemans, Hanneke C.Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands (författare)
  • Valent, Peter (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, AustriaMed Univ Vienna, Inst Environm Hlth, Vienna, Austria (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:The Lancet Haematology: ELSEVIER SCI LTD6:12, s. E638-E6492352-3026

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