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Anaphylaxis in Mastocytosis

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Abstract

Anaphylaxis is the most alarming systemic hypersensitivity reaction and can be fatal if not promptly treated. The diagnosis is often delayed, and misdiagnosis often occurs with asthma or urticaria and/or angioedema or vasovagal collapse. Existing evidence indicates a strong association between anaphylaxis and mastocytosis, as the prevalence of anaphylaxis among mastocytosis patients is clearly increased compared to that in the general population. Anaphylaxis in mastocytosis patients has special features, as severe cardiovascular signs and symptoms, for example, syncope, are the prominent findings. Hence, the presence of underlying mastocytosis should be further investigated in patients with severe anaphylactic reactions to hymenoptera venom, particularly men, or in patients presenting with recurrent, unexplained hypotensive syncopal episodes without urticaria or angioedema, especially if baseline tryptase levels are elevated (≥11.4 ng/mL). Appropriate treatment of acute anaphylaxis episodes with epinephrine is of paramount significance, as the delays in the treatment increase the risk of poor outcomes.

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Abbreviations

CM:

Cutaneous mastocytosis

ISM:

Indolent systemic mastocytosis

MC:

Mast cell

MIS:

Mastocytosis in the skin

MMAS:

Monoclonal mast cell activation syndrome

NSAIDs:

Nonsteroidal anti-inflammatory drugs

REMA:

Spanish Network on Mastocytosis

sBT:

Serum baseline tryptase

SM:

Systemic mastocytosis

VIA:

Venom-induced anaphylaxis

VIT:

Venom immunotherapy

WHO:

World Health Organization

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Authors and Affiliations

  1. Department of Respiratory Medicine and Allergic Diseases, Karolinska University Hospital, Huddinge, Sweden

    Theo Gülen

  2. Department of Medicine Solna, Immunology and Allergy Unit, Karolinska Institutet, Stockholm, Sweden

    Theo Gülen

  3. Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Joanne N. G. Oude Elberink

  4. Department of Dermatology and Allergology am Biederstein, Technical University of Munich, Munich, Germany

    Knut Brockow

Authors
  1. Theo Gülen
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  2. Joanne N. G. Oude Elberink
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  3. Knut Brockow
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Correspondence to Theo Gülen .

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Editors and Affiliations

  1. Professor of Medicine, Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI, USA

    Cem Akin

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Gülen, T., Oude Elberink, J.N.G., Brockow, K. (2020). Anaphylaxis in Mastocytosis. In: Akin, C. (eds) Mastocytosis. Springer, Cham. https://doi.org/10.1007/978-3-030-27820-5_9

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