Evidence map›Paper›PMID 30737190›Full record

ReviewThe journal of allergy and clinical immunology. In practice2019

Proposed Diagnostic Algorithm for Patients with Suspected Mast Cell Activation Syndrome.

Peter Valent, Cem Akin, Patrizia Bonadonna, Karin Hartmann, Knut Brockow, Marek Niedoszytko, Boguslaw Nedoszytko, Frank Siebenhaar, Wolfgang R Sperr, Joanna N G Oude Elberink and 14 more

Open access · greenAbstract readReview
In one paragraph

Review in The journal of allergy and clinical immunology. In practice, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 86 papers.

0numbers the graph read from it
0cells of the map it votes in
86citing papers in PubMed
12.2field-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

86 citing papers in PubMed, 202 citations in OpenAlex.

  1. Review
  2. Article
  3. Review
  4. Article
  5. Article
  6. Characteristics and outcomes of patients with pediatric-onset non-mastocytosis mast cell activation disorders: A CEREMAST study.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026
    Article
  7. Article
  8. Review
  9. Article
  10. Article
  11. Diagnosis and management of mast cell activation syndrome (MCAS) in Canada: a practical approach.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2025
    Review
  12. Article
  13. Review
  14. Review
  15. Mastocytosis.Nature reviews. Disease primers · 2025
    Review
  16. Article
  17. Review
  18. Article
  19. Article
  20. Review

26 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

24 authors at 20 institutions in 10 countries.

Peter ValentDepartment of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria. Electronic address: peter.valent@meduniwien.ac.at.
Cem AkinDivision of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Mich.
Patrizia BonadonnaAllergy Unit, Verona University Hospital, Verona, Italy.
Karin HartmannDepartment of Dermatology, University of Luebeck, Luebeck, Germany.
Knut BrockowDepartment of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
Marek NiedoszytkoDepartment of Allergology, Medical University of Gdansk, Gdansk, Poland.
Boguslaw NedoszytkoDepartment of Dermatology, Medical University of Gdansk, Gdansk, Poland.
Frank SiebenhaarDepartment of Dermatology & Allergy, Charité Universitätsmedizin Berlin, Berlin, Germany.
Wolfgang R SperrDepartment of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria.
Joanna N G Oude ElberinkDepartment of Allergology, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands.
Joseph H ButterfieldDivision of Allergic Diseases, Mayo Clinic, Rochester, Minn.
Ivan Alvarez-TwoseInstituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast) and CIBERONC, Hospital Virgen del Valle, Toledo, Spain.
Karl SotlarInstitute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria.
Andreas ReiterIII. Medizinische Klinik, Universitäts-Medizin Mannheim, Universität Heidelberg, Mannheim, Germany.
Hanneke C Kluin-NelemansDepartment of Haematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Olivier HermineImagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre national de référence des mastocytoses, Paris, France.
Jason GotlibStanford Cancer Institute/Stanford University School of Medicine, Stanford, Calif.
Sigurd Broesby-OlsenDepartment of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
Alberto OrfaoServicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL), IBSAL, CIBERONC and Department of Medicine, University of Salamanca, Salamanca, Spain.
Hans-Peter HornyInstitute of Pathology, Ludwig-Maximilian University, Munich, Germany.
Massimo TriggianiDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Michel ArockDepartment of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France.
Lawrence B SchwartzDepartment of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Virginia Commonwealth University, Richmond, Va.
Dean D MetcalfeLaboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
Gdańsk Medical University · PLMedical University of Vienna · ATUniversity Medical Center Groningen · NLCancer Institute (WIA) · INCentro de Investigación del Cáncer · ESCharité - Universitätsmedizin Berlin · DEHeidelberg University · DEHospital Virgen del Valle · ESLudwig-Maximilians-Universität München · DEMayo Clinic · USNational Institute of Allergy and Infectious Diseases · USOdense University Hospital · DKParacelsus Medical University · ATPitié-Salpêtrière Hospital · FRSorbonne Paris Cité · FRTechnical University of Munich · DEUniversity of Lübeck · DEUniversity of Michigan–Ann Arbor · USUniversity of Salerno · ITUniversity of Verona · IT

Funding

The Pathogenesis, Diagnosis, And Treatment Of Systemic Mast Cell DisordersZIAAI000249 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI KOMAROW, HIRSH D · 2009 to 2025
$16.5M
Pathogenesis of Physical Urticaria SyndromesZIAAI001091 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI KOMAROW, HIRSH D · 2009 to 2019
$3.8M
Intramural NIH HHS ZIA AI001091
6 · The paper itself

Abstract

Mast cell activation (MCA) accompanies diverse physiologic and pathologic processes and is one of the more frequently encountered conditions in medicine. MCA-related symptoms are usually mild and often transient. In such cases, histamine receptor blockers and other mediator-targeting drugs can usually control MCA. In severe cases, an MCA syndrome (MCAS) may be diagnosed. However, overt MCAS is an unusual condition, and many patients referred because of suspected MCAS are diagnosed with other diseases (autoimmune, neoplastic, or infectious) unrelated to MCA or suffer from MCA-related (eg, allergic) disorders and/or comorbidities without fulfilling criteria of an overt MCAS. These considerations are important as more and more patients are informed that they may have MCA or even MCAS without completing a thorough medical evaluation. In fact, in several instances, symptoms are misinterpreted as MCA/MCAS, and other clinically relevant conditions are not thoroughly pursued. The number of such referrals is increasing. To avoid such unnecessary referrals and to prevent misdiagnoses, we here propose a diagnostic algorithm through which a clinically relevant (systemic) MCA can be suspected and MCAS can subsequently be documented or excluded. In addition, the algorithm proposed should help guide the investigating care providers to consider the 2 principal diagnoses that may underlie MCAS, namely, severe allergy and systemic mastocytosis accompanied by severe MCA. Although validation is required, we anticipate that this algorithm will facilitate the management of patients with suspected MCAS.

Indexed as

AlgorithmsDiagnosis, DifferentialHumansHypersensitivity, ImmediateMastocytosisMastocytosis, SystemicProto-Oncogene Proteins c-kitTryptasesKIT protein, humanProto-Oncogene Proteins c-kitTryptasesDiagnostic algorithmKIT D816VMast cellsMCASTryptase

Identifiers

PMID30737190
PMCPMC6643056
OpenAlexW2914596877

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.