Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome.

Autor:	Valent P; Department of Internal Medicine I, Division of Hematology and Hemostaseology, and Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria, peter.valent@meduniwien.ac.at., Bonadonna P; Allergy Unit, Verona University Hospital, Verona, Italy., Hartmann K; Division of Allergy, Department of Dermatology, University of Basel, Basel, Switzerland., Broesby-Olsen S; Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark., Brockow K; Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany., Butterfield JH; Division of Allergic Diseases, Mayo Clinic, Rochester, Minnesota, USA., Triggiani M; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy., Lyons JJ; Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA., Oude Elberink JNG; Department of Allergology, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands., Arock M; Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), Paris, France., Metcalfe DD; Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, Maryland, USA., Akin C; Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, Michigan, USA.
Jazyk:	angličtina
Zdroj:	International archives of allergy and immunology [Int Arch Allergy Immunol] 2019; Vol. 180 (1), pp. 44-51. Date of Electronic Publication: 2019 Jun 28.
DOI:	10.1159/000501079
Abstrakt:	Mast cell activation syndrome (MCAS) is a condition characterized by recurrent episodes of clinically relevant, systemic, severe reactions to mast cell (MC)-derived mediators released in the context of anaphylaxis or another acute MC-related event. It is important to document MC involvement in these reactions in order to establish the diagnosis MCAS. The most specific and reliable marker of systemic MC activation is an acute and substantial event-related (transient) increase in the serum tryptase level over the individual's baseline value. However, the baseline level of tryptase varies depending on the underlying disease and the genetic background. For example, an estimated 3-5% of healthy individuals exhibit duplications or multiple copies of the TPSAB1 gene encoding for alpha-tryptase, and over 30% of all patients with myeloid neoplasms, including mastocytosis, have elevated basal tryptase levels. Therefore, it is of utmost importance to adjust the event-related diagnostic (MCAS-confirming) increase in tryptase over the individual baseline in a robust approach. To address this challenge, the 20% + 2 formula was proposed by the consensus group in 2012. Since then, this approach has been validated in clinical practice by independent groups and found to be sound. In the current article, we discuss the emerging importance and value of the 20% + 2 formula in clinical practice and its role as a criterion of severe systemic MC activation and MCAS. (© 2019 S. Karger AG, Basel.)
Databáze:	MEDLINE
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