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

Sperr, W. R., Kundi, M., Alvarez-Twose, I., van Anrooij, B., Elberink, J. N. G. O., Gorska, A., Niedoszytko, M., Gleixner, K. V., Hadzijusufovic, E., Zanotti, R., Bonadonna, P., Bonifacio, M., Perkins, C., Illerhaus, A., Elena, C., Merante, S., Shoumariyeh, K., von Bubnoff, N., Parente, R., Jawhar, M., Fortina, A. B., Caroppo, F., Brockow, K., Zink, A., Fuchs, D., Kilbertus, A. J., Yavuz, A. S., Doubek, M., Hagglund, H., Panse, J., Sabato, V., Bretterklieber, A., Niederwieser, D., Breynaert, C., Hartmann, K., Triggiani, M., Nedoszytko, B., Reiter, A., Orfao, A., Hermine, O., Gotlib, J., Arock, M., Kluin-Nelemans, H. C. & Valent, P., Dec-2019, In : Lancet Haematology. 6, 12, p. E638-E649 12 p.

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DOI

  • Wolfgang R. Sperr
  • Michael Kundi
  • Ivan Alvarez-Twose
  • Bjorn van Anrooij
  • Joanna N. G. Oude Elberink
  • Aleksandra Gorska
  • Marek Niedoszytko
  • Karoline V. Gleixner
  • Emir Hadzijusufovic
  • Roberta Zanotti
  • Patrizia Bonadonna
  • Massimiliano Bonifacio
  • Cecelia Perkins
  • Anja Illerhaus
  • Chiara Elena
  • Serena Merante
  • Khalid Shoumariyeh
  • Nikolas von Bubnoff
  • Roberta Parente
  • Mohamad Jawhar
  • Anna Belloni Fortina
  • Francesca Caroppo
  • Knut Brockow
  • Alexander Zink
  • David Fuchs
  • Alex J. Kilbertus
  • Akif Selim Yavuz
  • Michael Doubek
  • Hans Hagglund
  • Jens Panse
  • Vito Sabato
  • Agnes Bretterklieber
  • Dietger Niederwieser
  • Christine Breynaert
  • Karin Hartmann
  • Massimo Triggiani
  • Boguslaw Nedoszytko
  • Andreas Reiter
  • Alberto Orfao
  • Olivier Hermine
  • Jason Gotlib
  • Michel Arock
  • Hanneke C. Kluin-Nelemans
  • Peter Valent

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

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.

Original languageEnglish
Pages (from-to)E638-E649
Number of pages12
JournalLancet Haematology
Volume6
Issue number12
Publication statusPublished - Dec-2019

    Keywords

  • MAST-CELLS, KIT D816V, CLASSIFICATION, MUTATIONS, MIDOSTAURIN, SURVIVAL, EFFICACY, UTILITY, SAFETY, BURDEN

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