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

21st March 2025

Peter Valent, Cem Akin, Karin Hartmann, Ivan Alvarez-Twose, Knut Brockow, Olivier Hermine, Marek Niedoszytko, Juliana Schwaab, Jonathan J Lyons, Melody C Carter, Hanneke Oude Elberink, Joseph H Butterfield, Tracy I George, Georg Greiner, Celalettin Ustun, Patrizia Bonadonna, Karl Sotlar, Gunnar Nilsson, Mohamad Jawhar, Frank Siebenhaar, Sigurd Broesby-Olsen, Selim Yavuz, Roberta Zanotti, Magdalena Lange, Boguslaw Nedoszytko, Gregor Hoermann, Mariana Castells, Deepti H Radia, Javier I Muñoz-Gonzalez, Wolfgang R Sperr, Massimo Triggiani, Hanneke C Kluin-Nelemans, Stephen J Galli, Lawrence B Schwartz, Andreas Reiter, Alberto Orfao, Jason Gotlib, Michel Arock, Hans-Peter Horny, Dean D Metcalfe (2021). This article discusses Mastocytosis, and its classification into cutaneous, systemic, and MC sarcoma forms. It highlights the role of the KIT D816V mutation and other genetic markers in determining disease severity and prognosis. The article also proposes updated diagnostic criteria and a global classification system of MC disorders generally, including MCAS.

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