International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort studySperr, 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.
Research output: Contribution to journal › Article › Academic › peer-review
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.
|Number of pages||12|
|Publication status||Published - Dec-2019|
- MAST-CELLS, KIT D816V, CLASSIFICATION, MUTATIONS, MIDOSTAURIN, SURVIVAL, EFFICACY, UTILITY, SAFETY, BURDEN