World Health Organization Classification and Diagnosis of Mastocytosis: Update 2023 and Future Perspectives

Peter Valent; Karl Sotlar; Hans-Peter Horny; Michel Arock; Cem Akin

doi:10.1016/j.iac.2023.04.011

World Health Organization Classification and Diagnosis of Mastocytosis: Update 2023 and Future Perspectives

Immunol Allergy Clin North Am. 2023 Nov;43(4):627-649. doi: 10.1016/j.iac.2023.04.011. Epub 2023 Jul 18.

Authors

Peter Valent¹, Karl Sotlar², Hans-Peter Horny³, Michel Arock⁴, Cem Akin⁵

Affiliations

¹ Division of Hematology and Hemostaseology, Department of Internal Medicine I, Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Wäheringer Gürtel 18-20, A-1090 Vienna, Austria. Electronic address: peter.valent@meduniwien.ac.at.
² Institute of Pathology, Paracelsus Medical University Salzburg, Austria.
³ Institute of Pathology, Paracelsus Medical University Salzburg, Austria; Institute of Pathology, Ludwig Maximilians University, Munich, Germany.
⁴ Department of Hematological Biology, Pitié-Salpêtrière Hospital, DMU BioGem, AP-HP.Sorbonne University, Paris, France; Platform of Molecular Analysis for Mastocytosis and Mast Cell Activation Syndromes (MCAS), Saint-Antoine Hospital, DMU BioGem, AP-HP.Sorbonne University, Paris, France.
⁵ Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI, USA.

PMID: 37758403
DOI: 10.1016/j.iac.2023.04.011

Abstract

Experts of the European Competence Network on Mastocytosis (ECNM) and the American Initiative on Mast Cell Disorders have discussed and updated diagnostic criteria and the classification of mastocytosis, based on new insights in the field and data collected in recent years, mostly within ECNM registry projects in which studies on several thousand cases have been performed. Based on this proposal, the World Health Organization has updated its classification of mastocytosis. This article discusses the revised classification of mastocytosis in light of a rapidly moving field and the advent of new diagnostic parameters, new prognostication tools, and new therapies.

Keywords: Diagnostic criteria; ICC; Mast cells; Mastocytosis; WHO classification.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Humans
Mast Cells
Mastocytosis* / diagnosis
Mastocytosis* / genetics
Mastocytosis* / therapy
World Health Organization