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Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria

Peter Valent, Cem Akin, Luís Escribano, Manuela Födinger, Karin Hartmann, Knut Brockow, Mariana Castells, Wolfgang R. Sperr, Hanneke C. Kluin‐Nelemans, Neveen A. T. Hamdy, Olivier Lortholary, J. Robyn, J. van Doormaal, Karl Sotlar, Alexander W. Hauswirth, Michel Arock, Olivier Hermine, Andrzej Rafal Hellmann, Massimo Triggiani, Marek Niedoszytko, Lawrence B. Schwartz, A. Orfao, H.‐P. Horny, Dean D. Metcalfe

📄 Abstract

Although a classification for mastocytosis and diagnostic criteria are available, there remains a need to define standards for the application of diagnostic tests, clinical evaluations, and treatment responses. To address these demands, leading experts discussed current issues and standards in mastocytosis in a Working Conference. The present article provides the resulting outcome with consensus statements, which focus on the appropriate application of clinical and laboratory tests, patient selection for interventional therapy, and the selection of appropriate drugs. In addition, treatment response criteria for the various clinical conditions, disease-specific symptoms, and specific pathologies are provided. Resulting recommendations and algorithms should greatly facilitate the management of patients with mastocytosis in clinical practice, selection of patients for therapies, and the conduct of clinical trials.

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