Evidence map›Paper›PMID 34901755›Full record

ReviewHemaSphere2021

Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal.

Peter Valent, Cem Akin, Karin Hartmann, Ivan Alvarez-Twose, Knut Brockow, Olivier Hermine, Marek Niedoszytko, Juliana Schwaab, Jonathan J Lyons, Melody C Carter and 30 more

Open access · goldAbstract readReview
In one paragraph

Review in HemaSphere, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 143 papers.

0numbers the graph read from it
0cells of the map it votes in
143citing papers in PubMed
16.4field-weighted citation impact, top 1% 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

143 citing papers in PubMed, 280 citations in OpenAlex.

  1. Psychometric evaluation of the Mastocytosis Symptom Severity Daily Diary (MS2D2) in adults with nonadvanced systemic mastocytosis (NonAdvSM).Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation · 2026
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  12. Development and validation of a quality of life scale for pediatric mastocytosis.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2026
    Article
  13. Review
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  15. Article
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  19. Diagnosing Systemic Mastocytosis: State of the Art.International journal of laboratory hematology · 2026
    Review
  20. Article

83 more citing papers are in PubMed but not listed here.

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

40 authors at 20 institutions in 12 countries.

Peter ValentDivision of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria.
Cem AkinDivision of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Michigan, USA.
Karin HartmannDivision of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Switzerland.
Ivan Alvarez-TwoseInstituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain.
Knut BrockowDepartment of Dermatology and Allergy Biederstein, Technical University of Munich, Germany.
Olivier HermineImagine Institute Université de Paris, Sorbonne, Institut national de la santé et de la recherche médicale U1163, Centre national de référence des mastocytoses, Hôpital Necker, Assistance publique hôpitaux de Paris, France.
Marek NiedoszytkoDepartment of Allergology, Medical University of Gdansk, Poland.
Juliana SchwaabDepartment of Hematology and Oncology, University Hospital Mannheim, Germany.
Jonathan J LyonsTranslational Allergic Immunopathology Unit, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.
Melody C CarterMast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.
Hanneke Oude ElberinkDepartment of Allergology, University Medical Center Groningen and GRIAC Research Institute, University of Groningen, The Netherlands.
Joseph H ButterfieldDivision of Allergic Diseases, Mayo Clinic, Rochester, Minnesota, USA.
Tracy I GeorgeDepartment of Pathology, University of Utah, Salt Lake City, Utah, USA.
Georg GreinerLudwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.
Celalettin UstunDivision of Hematology, Oncology and Cell Therapy, Department of Medicine, The Coleman Foundation Blood and Marrow Transplant Center at Rush University Medical Center, Chicago, Illinois, USA.
Patrizia BonadonnaAllergy Unit, Verona University Hospital, Verona, Italy.
Karl SotlarInstitute of Pathology, Paracelsus Medical University Salzburg, Austria.
Gunnar NilssonDepartment of Medicine Solna & Mastocytosis Centre, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden.
Mohamad JawharDepartment of Hematology and Oncology, University Hospital Mannheim, Germany.
Frank SiebenhaarDermatological Allergology, Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt - Universität zu Berlin, and Berlin Institute of Health, Germany.
Sigurd Broesby-OlsenDepartment of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
Selim YavuzDivision of Haematology, Department of Internal Medicine, University of Istanbul, Turkey.
Roberta ZanottiSection of Hematology, Multidisciplinary Outpatients Clinics for Mastocytosis, Department of Medicine, University Hospital of Verona, Italy.
Magdalena LangeDepartment of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland.
Boguslaw NedoszytkoDepartment of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland.
Gregor HoermannLudwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Austria.
Mariana CastellsDivision of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Deepti H RadiaDepartment of Clinical Haematology, Guys and St Thomas' NHS Hospitals, London, United Kingdom.
Javier I Muñoz-GonzalezServicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain.
Wolfgang R SperrDivision of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Austria.
Massimo TriggianiDivision of Allergy and Clinical Immunology, University of Salerno, Italy.
Hanneke C Kluin-NelemansDepartment of Haematology, University Medical Center Groningen, University of Groningen, The Netherlands.
Stephen J GalliDepartment of Pathology and the Sean N. Parker Center for Allergy and Asthma Research, Stanford University School of Medicine, Stanford, California, USA.
Lawrence B SchwartzDivision of Rheumatology, Allergy & Immunology, Department of Internal Medicine, Virginia Commonwealth University (VCU), Richmond, Virginia, USA.
Andreas ReiterDepartment of Hematology and Oncology, University Hospital Mannheim, Germany.
Alberto OrfaoServicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) Instituto Biosanitario de Salamanca (IBSAL) and Department of Medicine, University of Salamanca, Spain.
Jason GotlibStanford Cancer Institute/Stanford University School of Medicine/Stanford Cancer Institute, Stanford, California, USA.
Michel ArockDepartment of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France.
Hans-Peter HornyInstitute of Pathology, Paracelsus Medical University Salzburg, Austria.
Dean D MetcalfeMast Cell Biology Section, Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA.
Medical University of Vienna · ATGdańsk Medical University · PLTechnical University of Munich · DEUniversity of Michigan–Ann Arbor · USHeidelberg University · DEHospital Virgen del Valle · ESNational Institute of Allergy and Infectious Diseases · USUniversity Hospital of Basel · CHUniversity of Basel · CHBrigham and Women's Hospital · USCharité - Universitätsmedizin Berlin · DEInstitut des Maladies Génétiques Imagine · FRInstituto de Investigación Biomédica de Salamanca · ESKarolinska University Hospital · SEOdense University Hospital · DKSorbonne Université · FRStanford University · USUniversidad de Salamanca · ESUniversity Medical Center Groningen · NLUniversity Medical Centre Mannheim · DE

Funding

Austrian Science Fund FWF P 32470
6 · The paper itself

Abstract

Mastocytosis is a hematologic neoplasm characterized by expansion and focal accumulation of neoplastic mast cells (MC) in diverse organs, including the skin, bone marrow (BM), spleen, liver, and gastrointestinal tract. The World Health Organization classification divides the disease into prognostically distinct variants of cutaneous mastocytosis (CM) and systemic mastocytosis (SM). Although this classification remains valid, recent developments in the field and the advent of new diagnostic and prognostic parameters created a need to update and refine definitions and diagnostic criteria in MC neoplasms. In addition, MC activation syndromes (MCAS) and genetic features predisposing to SM and MCAS have been identified. To discuss these developments and refinements in the classification, we organized a Working Conference comprised of experts from Europe and the United States in August 2020. This article reports on outcomes from this conference. Of particular note, we propose adjustments in the classification of CM and SM, refinements in diagnostic criteria of SM variants, including smoldering SM and BM mastocytosis (BMM), and updated criteria for MCAS and other conditions involving MC. CD30 expression in MC now qualifies as a minor SM criterion, and BMM is now defined by SM criteria, absence of skin lesions and absence of B- and C-findings. A basal serum tryptase level exceeding 20 ng/mL remains a minor SM criterion, with recognition that hereditary alpha-tryptasemia and various myeloid neoplasms may also cause elevations in tryptase. Our updated proposal will support diagnostic evaluations and prognostication in daily practice and the conduct of clinical trials in MC disorders.

Identifiers

PMID34901755
PMCPMC8659997
OpenAlexW3206166311

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.