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@article{1617518,
author = {Sperr, W.R. and Kundi, M. and AlvarezandTwose, I. and van Anrooij, B. and Elberink, J.N.G.O. and Gorska, A. and Niedoszytko, M. and Gleixner, K.V. and Hadzijusufovic, E. and Zanotti, R. and Bonadonna, P. and Bonifacio, M. and Perkins, C. and Illerhaus, A. and Elena, C. and Merante, S. and Shoumariyeh, K. and von Bubnoff, N. and Parente, R. and Jawhar, M. and Fortina, A.B. and Caroppo, F. and Brockow, K. and Zink, A. and Fuchs, D. and Kilbertus, A.J. and Yavuz, A.S. and Doubek, Michael and Hagglund, H. and Panse, J. and Sabato, V. and Bretterklieber, A. and Niederwieser, D. and Breynaert, C. and Hartmann, K. and Triggiani, M. and Nedoszytko, B. and Reiter, A. and Orfao, A. and Hermine, O. and Gotlib, J. and Arock, M. and KluinandNelemans, H.C. and Valent, P.},
article_location = {England},
article_number = {12},
doi = {http://dx.doi.org/10.1016/S2352-3026(19)30166-8},
keywords = {MAST-CELLS; KIT D816V; CLASSIFICATION; MUTATIONS; MIDOSTAURIN; SURVIVAL; EFFICACY; UTILITY; SAFETY; BURDEN},
language = {eng},
issn = {2352-3026},
journal = {The Lancet. Haematology},
title = {International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study},
url = {https://www.sciencedirect.com/science/article/abs/pii/S2352302619301668?via%3Dihub#!},
volume = {6},
year = {2019}
}
TY - JOUR
ID - 1617518
AU - 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, Michael - 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.
PY - 2019
TI - International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study
JF - The Lancet. Haematology
VL - 6
IS - 12
SP - "E638"-"E649"
EP - "E638"-"E649"
PB - ELSEVIER SCI LTD
SN - 23523026
KW - MAST-CELLS
KW - KIT D816V
KW - CLASSIFICATION
KW - MUTATIONS
KW - MIDOSTAURIN
KW - SURVIVAL
KW - EFFICACY
KW - UTILITY
KW - SAFETY
KW - BURDEN
UR - https://www.sciencedirect.com/science/article/abs/pii/S2352302619301668?via%3Dihub#!
L2 - https://www.sciencedirect.com/science/article/abs/pii/S2352302619301668?via%3Dihub#!
N2 - 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.
ER -
SPERR, W.R., M. KUNDI, I. ALVAREZ-TWOSE, B. VAN ANROOIJ, J.N.G.O. ELBERINK, A. GORSKA, M. NIEDOSZYTKO, K.V. GLEIXNER, E. HADZIJUSUFOVIC, R. ZANOTTI, P. BONADONNA, M. BONIFACIO, C. PERKINS, A. ILLERHAUS, C. ELENA, S. MERANTE, K. SHOUMARIYEH, N. VON BUBNOFF, R. PARENTE, M. JAWHAR, A.B. FORTINA, F. CAROPPO, K. BROCKOW, A. ZINK, D. FUCHS, A.J. KILBERTUS, A.S. YAVUZ, Michael DOUBEK, H. HAGGLUND, J. PANSE, V. SABATO, A. BRETTERKLIEBER, D. NIEDERWIESER, C. BREYNAERT, K. HARTMANN, M. TRIGGIANI, B. NEDOSZYTKO, A. REITER, A. ORFAO, O. HERMINE, J. GOTLIB, M. AROCK, H.C. KLUIN-NELEMANS a P. VALENT. International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study. \textit{The Lancet. Haematology}. England: ELSEVIER SCI LTD, 2019, roč.~6, č.~12, s.~''E638''-''E649'', 12 s. ISSN~2352-3026. Dostupné z: https://dx.doi.org/10.1016/S2352-3026(19)30166-8.
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