Evaluation of mast cell activation syndromes: impact of pathology and immunohistology

International Archives of Allergy and Immunology
H-P HornyPeter Valent

Abstract

Mast cell activation syndromes (MCAS) are clinically defined disease states with a largely unknown morphological background. Since mastocytosis may be associated with MCAS, it is crucial in every patient to document or exclude mastocytosis by appropriate histological, molecular, and serological investigations of tissues/organs that are commonly involved in mastocytosis like skin, mucosa of the gastrointestinal tract and bone marrow. Accordingly, histopathological investigation including immunohistological stains is crucial to reach the final diagnosis in such patients and to classify MCAS into primary MCAS, which can present with or without evidence of overt mastocytosis, or secondary MCAS, where an underlying disease with or without tissue inflammation is detected. Cases without evidence of mastocytosis, monoclonal mast cells, or any underlying disease should be termed idiopathic MCAS. When the activating point mutant KIT D816V is detectable but criteria for diagnosis of mastocytosis are not completely met, a so-called (mono)clonal MCAS as a subvariant of primary MCAS should be diagnosed.

References

May 30, 2001·Leukemia Research·P ValentJ M Bennett
Jun 26, 2007·Pathobiology : Journal of Immunopathology, Molecular and Cellular Biology·Hans-Peter HornyPeter Valent
Jan 13, 2009·The Journal of Allergy and Clinical Immunology·Patrizia BonadonnaRoberta Zanotti
Mar 14, 2009·The Journal of Allergy and Clinical Immunology·Dean D Metcalfe, Lawrence B Schwartz
May 4, 2010·The Journal of Allergy and Clinical Immunology·Iván Alvarez-TwoseLuis Escribano
Jul 10, 2010·Pathobiology : Journal of Immunopathology, Molecular and Cellular Biology·H-P HornyP Valent
Nov 3, 2010·The Journal of Allergy and Clinical Immunology·Cem AkinDean D Metcalfe

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Citations

Feb 7, 2013·Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete·K Brockow
Dec 6, 2012·Current Allergy and Asthma Reports·Juan-Carlos CardetMatthew J Hamilton
May 7, 2015·Clinical Reviews in Allergy & Immunology·Marianne Frieri
Oct 4, 2016·International Immunopharmacology·Jatinder SinghDhandeep Singh
May 3, 2014·Expert Review of Clinical Immunology·Anastasia I PetraTheoharis C Theoharides
Aug 16, 2015·American Journal of Clinical Pathology·Kaaren K ReichardCurtis A Hanson
Jan 10, 2018·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Nicola Wagner, Petra Staubach
Jan 10, 2018·Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG·Nicola Wagner, Petra Staubach
Mar 19, 2019·Expert Review of Clinical Immunology·Theoharis C TheoharidesHuali Ren

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