Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock

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Casassa, Eline A. | Mailhol, Claire | Tournier, Emilie | Laurent, Camille | Degboe, Yannick | Eischen, Marine | Kirsten, Natalia | Moreau, Jacques | Evrard, Solène M. | Mansat-de Mas, Véronique | Lamant, Laurence | Dubreuil, Patrice | Apoil, Pol-André | Hermine, Olivier | Paul, Carle | Bulai Livideanu, Cristinai

Edité par CCSD ; Wiley -

International audience. Mast cell activation syndrome (MCAS), is associated with mast cell activation-related symptoms involving two or more organs. The disease in patients with MCAS does not fulfill the diagnostic criteria for systemic (SM) or cutaneous mastocytosis. There is limited information about the clinical and biological characteris-tics of MCAS in clinical practice.1,2,4 The performance of the proposed diagnostic criteria for MCAS has not been prospectively assessed. Bone marrow tryptase has been shown to be a sensitive diagnostic tool for SM even in patients with normal serum tryptase (ST).The primary objective of this study was to describe the clinicaland paraclinical characteristics of a cohort of patients with primary MCAS. The secondary objective was to describe the usability of the two available sets of diagnostic criteria for MCAS. We included all consecutive adult patients with a diagnosis of primary MCAS evaluated between April 2011 and May 2015. The diagnostic of MCAS was established using the diagnostic criteria defined by Valent et al. and/or those defined by Molderings et al. The presence/absence of a monoclonal mast cell carrying the D816V KIT mutation and/or expression of CD25 in bone marrow determined the classification of patients as monoclonal MCAS (MMCAS)/idiopathic MCAS (IMCAS) respectively.

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