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Allergy. 2015 Jul;70(7):755-63. doi: 10.1111/all.12617. Epub 2015 Apr 24.

Drug hypersensitivity in clonal mast cell disorders: ENDA/EAACI position paper.

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Allergy Unit, Azienda Ospedaliera Universitaria Intergrata of Verona, Verona, Italy.
Medicine Ward, Pieve di Coriano Hospital Azienda Ospedaliera Carlo Poma Mantova, Mantova, Italy.
Department of Dermatology and Venerology, Medizinische Universitat Graz, Graz, Austria.
Allergy Unit, Department of Internal Medicine, Ospedali Riuniti di Ancona, Ancona, Italy.
Department of Dermatology and Allergy Biederstein, Technische Universität München, Munich, Germany.
Department of Allergology, GRIAC Research Institute, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Allergy Clinic, Copenhagen University Hospital, Gentofte, Denmark.
Allergy Unit Complesso Integrato Columbus, Rome and IRCCS Oasi Maria S.S., Troina, Italy.
Department of Medicine, Hematology Section, Azienda Ospedaliera Universitaria Intergrata of Verona, Verona, Italy.
Allergy Unit, Regional University Hospital-IBIMA, UMA, Málaga, Spain.


Mastocytosis is a clonal disorder characterized by the proliferation and accumulation of mast cells (MC) in different tissues, with a preferential localization in skin and bone marrow (BM). The excess of MC in mastocytosis as well as the increased releasability of MC may lead to a higher frequency and severity of immediate hypersensitivity reactions. Mastocytosis in adults is associated with a history of anaphylaxis in 22-49%. Fatal anaphylaxis has been described particularly following hymenoptera stings, but also occasionally after the intake of drugs such as nonsteroidal anti-inflammatory drugs, opioids and drugs in the perioperative setting. However, data on the frequency of drug hypersensitivity in mastocytosis and vice versa are scarce and evidence for an association appears to be limited. Nevertheless, clonal MC disorders should be ruled out in cases of severe anaphylaxis: basal serum tryptase determination, physical examination for cutaneous mastocytosis lesions, and clinical characteristics of anaphylactic reaction might be useful for differential diagnosis. In this position paper, the ENDA group performed a literature search on immediate drug hypersensitivity reactions in clonal MC disorders using MEDLINE, EMBASE, and Cochrane Library, reviewed and evaluated the literature in five languages using the GRADE system for quality of evidence and strength of recommendation.


clonal mast cell disorders; drug allergy; drug hypersensitivity; mastocytosis; tryptase

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