Sperr, Wolfgang R., Kundi, Michael, Alvarez-Twose, Ivan, van Anrooij, Bjorn, Elberink, Joanna N. G. Oude, Gorska, Aleksandra ORCID: 0000-0001-6682-1555, Niedoszytko, Marek ORCID: 0000-0003-1089-1911, Gleixner, Karoline V., Hadzijusufovic, Emir ORCID: 0000-0001-7409-4204, Zanotti, Roberta, Bonadonna, Patrizia, Bonifacio, Massimiliano ORCID: 0000-0003-0716-1686, Perkins, Cecelia, Illerhaus, Anja, Elena, Chiara, Merante, Serena, Shoumariyeh, Khalid ORCID: 0000-0001-7969-1972, von Bubnoff, Nikolas, Parente, Roberta, Jawhar, Mohamad, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Caroppo, Francesca, Brockow, Knut, Zink, Alexander ORCID: 0000-0001-9313-6588, Fuchs, David, Kilbertus, Alex J., Yavuz, Akif Selim, Doubek, Michael, Hagglund, Hans, Panse, Jens, Sabato, Vito, Bretterklieber, Agnes, Niederwieser, Dietger, Breynaert, Christine, Hartmann, Karin ORCID: 0000-0002-4595-8226, Triggiani, Massimo, Nedoszytko, Boguslaw, Reiter, Andreas, Orfao, Alberto, Hermine, Olivier, Gotlib, Jason, Arock, Michel, Kluin-Nelemans, Hanneke C. and Valent, Peter ORCID: 0000-0003-0456-5095 (2019). International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study. Lancet Haematol., 6 (12). S. E638 - 12. OXFORD: ELSEVIER SCI LTD. ISSN 2352-3026

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Abstract

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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kundi, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Alvarez-Twose, IvanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjornUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elberink, Joanna N. G. OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDorcid.org/0000-0001-6682-1555UNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDorcid.org/0000-0003-1089-1911UNSPECIFIED
Gleixner, Karoline V.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hadzijusufovic, EmirUNSPECIFIEDorcid.org/0000-0001-7409-4204UNSPECIFIED
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonifacio, MassimilianoUNSPECIFIEDorcid.org/0000-0003-0716-1686UNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Merante, SerenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDorcid.org/0000-0001-7969-1972UNSPECIFIED
von Bubnoff, NikolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Fuchs, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kilbertus, Alex J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bretterklieber, AgnesUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niederwieser, DietgerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Breynaert, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nedoszytko, BoguslawUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Orfao, AlbertoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDorcid.org/0000-0003-0456-5095UNSPECIFIED
URN: urn:nbn:de:hbz:38-126279
DOI: 10.1016/S2352-3026(19)30166-8
Journal or Publication Title: Lancet Haematol.
Volume: 6
Number: 12
Page Range: S. E638 - 12
Date: 2019
Publisher: ELSEVIER SCI LTD
Place of Publication: OXFORD
ISSN: 2352-3026
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAST-CELLS; KIT D816V; CLASSIFICATION; MUTATIONS; MIDOSTAURIN; SURVIVAL; EFFICACY; UTILITY; SAFETY; BURDENMultiple languages
HematologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/12627

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