Evidence map›Paper›PMID 34545185›Full record

ArticleLeukemia2022

Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis.

Roberta Zanotti, Massimiliano Bonifacio, Giuseppe Lucchini, Wolfgang R Sperr, Luigi Scaffidi, Björn van Anrooij, Hanneke Nc Oude Elberink, Julien Rossignol, Olivier Hermine, Aleksandra Gorska and 38 more

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Article in Leukemia, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.

0numbers the graph read from it
0cells of the map it votes in
24citing papers in PubMed
3.5field-weighted citation impact, top 6% of its field
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

24 citing papers in PubMed, 53 citations in OpenAlex.

  1. Review
  2. Review
  3. Article
  4. Article
  5. Article
  6. Review
  7. Mastocytosis.Nature reviews. Disease primers · 2025
    Review
  8. Review
  9. Article
  10. Article
  11. Article
  12. Review
  13. Review
  14. Cutaneous Mastocytosis in Childhood: An Update from the Literature.Journal of clinical practice and research · 2023
    Review
  15. Article
  16. Article
  17. Article
  18. Article
  19. Review
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

48 authors at 20 institutions in 13 countries.

Roberta ZanottiDepartment of Medicine, Section of Hematology, University of Verona, Verona, Italy.
Massimiliano BonifacioDepartment of Medicine, Section of Hematology, University of Verona, Verona, Italy. massimiliano.bonifacio@univr.it.ORCID http://orcid.org/0000-0003-0716-1686
Giuseppe LucchiniBiostatistical Service ASST of Mantova, Mantua, Italy.
Wolfgang R SperrDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.
Luigi ScaffidiDepartment of Medicine, Section of Hematology, University of Verona, Verona, Italy.
Björn van AnrooijDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Hanneke Nc Oude ElberinkDepartment of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Julien RossignolInstitut Imagine INSERM Unité 1163 and Centre National de la Recherche Scientifique ERL8654, Centre de Reference des Mastocytoses, University of Paris, Paris, France.
Olivier HermineInstitut Imagine INSERM Unité 1163 and Centre National de la Recherche Scientifique ERL8654, Centre de Reference des Mastocytoses, University of Paris, Paris, France.
Aleksandra GorskaDepartment of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Magdalena LangeDepartment of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Emir HadzijusufovicDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.ORCID http://orcid.org/0000-0001-7409-4204
Cornelius MiethingDepartment of Medicine I, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Germany and German Cancer Consortium (DKTK), Partner Site Freiburg, Freiburg, Germany.ORCID http://orcid.org/0000-0003-4699-3805
Sabine MüllerDepartment of Medicine I, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Germany and German Cancer Consortium (DKTK), Partner Site Freiburg, Freiburg, Germany.
Cecelia PerkinsDivision of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
William ShomaliDivision of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
Chiara ElenaDivision of Hematology, Fondazione IRCCS San Matteo, Pavia, Italy.
Anja IllerhausDepartment of Dermatology, University of Cologne, Cologne, Germany.
Mohamad JawharHämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Roberta ParenteDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Francesca CaroppoPediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.ORCID http://orcid.org/0000-0003-3583-0816
Oleksii SolomianyiDepartment of Internal Medicine 3, Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Alexander ZinkDepartment of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
Mattias MattssonDepartment of Medical Sciences, Uppsala University and Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
Akif Selim YavuzDivision of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
Jens PanseDepartment of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany.
Judit VarkonyiDepartment of Hematology and Internal Medicine, Semmelweis University, Budapest, Hungary.
Michael DoubekUniversity Hospital, Department of Hematology and Oncology, and Department of Medical Genetics and Genomics, Faculty of Medicine Masaryk University, Brno, Czech Republic.ORCID http://orcid.org/0000-0002-1269-6282
Vito SabatoFaculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.
Christine BreynaertKU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium.
Vladan VucinicMedical Clinic and Policlinic 1, Hematology and Cellular Therapy, Leipzig University Hospital, Leipzig, Germany.
Tanja SchugDepartment of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
Hans HägglundDepartment of Medical Sciences, Uppsala University and Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
Friederike WortmannDepartment of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Lübeck, Germany.
Knut BrockowDepartment of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
Irena Angelova-FischerDepartment of Dermatology and Venerology (AK), Kepler University Hospital, Johannes Kepler University, Linz, Austria.
Anna Belloni FortinaPediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy.
Massimo TriggianiDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Andreas ReiterHämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Karin HartmannDivision of Allergy, Department of Dermatology, and Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland.
Luca MalcovatiDivision of Hematology, Fondazione IRCCS San Matteo, Pavia, Italy.ORCID http://orcid.org/0000-0002-1460-1611
Jason GotlibDivision of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA.
Khalid ShoumariyehDepartment of Medicine I, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Germany and German Cancer Consortium (DKTK), Partner Site Freiburg, Freiburg, Germany.
Marek NiedoszytkoDepartment of Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Michel ArockLaboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France.
Hanneke C Kluin-NelemansDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Patrizia BonadonnaAllergy Unit, Verona University Hospital, Verona, Italy.
Peter ValentDepartment of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.ORCID http://orcid.org/0000-0003-0456-5095
University of Verona · ITGdańsk Medical University · PLStanford Cancer InstituteUniversity Medical Center Groningen · NLUniversity of Freiburg · DEHeidelberg University · DEInserm · FRMedical University of Vienna · ATTechnical University of Munich · DEUniversity of Padua · ITUniversity of Salerno · ITUppsala University Hospital · SEIstanbul University · TRJohannes Kepler University of Linz · ATKU Leuven · BEMasaryk University · CZMedical University of Graz · ATPoliclinico San Matteo Fondazione · ITRWTH Aachen University · DESemmelweis University · HU

Funding

Austrian Science Fund FWF P 32470
6 · The paper itself

Abstract

In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.

Indexed as

AdultAgedAged, 80 and overBone MarrowEuropeFemaleFollow-Up StudiesHumansMaleMast CellsMastocytosisMastocytosis, SystemicMiddle AgedPrognosisSkin DiseasesSurvival RateTryptases

Identifiers

PMID34545185
OpenAlexW3201033674

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