Evidence map›Paper›PMID 31676322›Full record

ArticleThe Lancet. Haematology2019

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

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 and 34 more

Abstract readValidation Study
PubMed Full text at PMC Publisher
In one paragraph

Article in The Lancet. Haematology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 75 papers.

0numbers the graph read from it
0cells of the map it votes in
75citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

75 citing papers in PubMed.

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  18. Mastocytosis.Nature reviews. Disease primers · 2025
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15 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

44 authors.

Wolfgang R SperrDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria. Electronic address: wolfgang.r.sperr@meduniwien.ac.at.
Michael KundiInstitute of Environmental Health, Medical University of Vienna, Vienna, Austria.
Ivan Alvarez-TwoseInstituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo, Spain.
Bjorn van AnrooijDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Joanna N G Oude ElberinkDepartment of Allergology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Aleksandra GorskaDepartment of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Marek NiedoszytkoDepartment of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Karoline V GleixnerDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
Emir HadzijusufovicDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria; Internal Medicine Small Animals, University Clinic for Small Animals, Department/University Clinic for Companion Animals and Horses, University of Veterinary Medicine, Vienna, Austria.
Roberta ZanottiSection of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.
Patrizia BonadonnaAllergy Unit, Verona University Hospital, Verona, Italy.
Massimiliano BonifacioSection of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.
Cecelia PerkinsDivision of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA.
Anja IllerhausDepartment of Dermatology, University of Cologne, Cologne, Germany.
Chiara ElenaDepartment of Molecular Medicine and Department of Hematology Oncology, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Serena MeranteDepartment of Molecular Medicine and Department of Hematology Oncology, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Khalid ShoumariyehDepartment of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Nikolas von BubnoffDepartment of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
Roberta ParenteDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Mohamad JawharIII Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Anna Belloni FortinaPediatric Dermatology Unit, Department of Medicine, University of Padua, Padua, Italy.
Francesca CaroppoPediatric Dermatology Unit, Department of Medicine, University of Padua, Padua, Italy.
Knut BrockowDepartment of Dermatology and Allergy, School of Medicine, Technical University of Munich, Munich, Germany.
Alexander ZinkDepartment of Dermatology and Allergy, School of Medicine, Technical University of Munich, Munich, Germany.
David FuchsUniversity Clinic for Hematology and Internal Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Alex J KilbertusDepartment of Dermatology and Venerology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Akif Selim YavuzDivision of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
Michael DoubekUniversity Hospital and CEITEC Masaryk University, Brno, Czech Republic.
Hans HägglundDivision of Hematology, Department of Medical Sciences Uppsala University, Uppsala, Sweden.
Jens PanseDepartment of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
Vito SabatoFaculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.
Agnes BretterklieberDepartment of Dermatology and Venereology, University Hospital Graz, Graz, Austria.
Dietger NiederwieserUniversity Hospital of Leipzig, Leipzig, Germany.
Christine BreynaertKU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium.
Karin HartmannDepartment of Dermatology, University of Cologne, Cologne, Germany; Division of Allergy, Department of Dermatology, and Department of Biomedicine, University of Basel, Basel, Switzerland.
Massimo TriggianiDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Boguslaw NedoszytkoDepartment of Dermatology, Medical University of Gdańsk, Gdańsk, Poland.
Andreas ReiterIII Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Alberto OrfaoCentro de Investigación del Cáncer/IBMCC (USAL/CSIC), CIBERONC and IBSAL, Departamento de Medicina and Servicio General de Citometría, University of Salamanca, Salamanca, Spain.
Olivier HermineImagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre national de référence des mastocytoses, Paris, France.
Jason GotlibDivision of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA.
Michel ArockDepartment of Hematological Biologie, Pitié-Salpêtrière Hospital, Paris Sorbonne University, Paris UMR8113, Ecole, France.
Hanneke C Kluin-NelemansDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Peter ValentDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.

Funding

Austrian Science Fund FWF F 4701Austrian Science Fund FWF F 4704
6 · The paper itself

Abstract

backgroundThe 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.

methodsWe 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 Española de Mastocitosis diagnosed between Jan 22, 1998, and Nov 2, 2017.

findingsThe 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 × 10

interpretationThe 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.

fundingAustrian Science Fund, Deutsche Forschungsgemeinschaft, Koeln Fortune Program, Charles and Ann Johnson Foundation, Instituto de Salud Carlos III, Fondos FEDER, Research-Foundation Flanders/Fonds Wetenschappelijk Onderzoek, Clinical Research-Fund of the University Hospitals Leuven, and Research-Foundation Flanders/Fonds Wetenschappelijk Onderzoek.

Indexed as

AdolescentAdultAgedAged, 80 and overCohort StudiesFemaleHumansInternationalityMaleMastocytosisMiddle AgedPrognosisRegistriesResearch DesignRetrospective StudiesSpain

Identifiers

PMID31676322
PMCPMC7115823

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