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Anaphylaxis as a Clinical Manifestation of Clonal Mast Cell Disorders

  • ANAPHYLAXIS AND DRUG ALLERGY (DA KHAN AND M CASTELLS, SECTION EDITORS)
  • Published:
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Abstract

Clonal mast cell disorders comprise a heterogeneous group of disorders characterized by the presence of gain of function KIT mutations and a constitutively altered activation-associated mast cell immunophenotype frequently associated with clinical manifestations related to the release of mast cells mediators. These disorders do not always fulfil the World Health Organization (WHO)-proposed criteria for mastocytosis, particularly when low-sensitive diagnostic approaches are performed. Anaphylaxis is a frequent presentation of clonal mast cell disorders, particularly in mastocytosis patients without typical skin lesions. The presence of cardiovascular symptoms, e.g., hypotension, occurring after a hymenoptera sting or spontaneously in the absence of cutaneous manifestations such as urticaria is characteristic and differs from the presentation of anaphylaxis in the general population without mastocytosis.

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Abbreviations

AAAAI:

American Academy of Allergy Asthma and Immunology

ACAAI:

American College of Allergy Asthma and Immunology

ASM:

Aggressive systemic mastocytosis

BM:

Bone marrow

BM MC:

Bone marrow mast cells

c-MCD:

Clonal mast cell disorders

c-MACD:

Clonal mast cell activation disorders

CM:

Cutaneous mastocytosis

ISM:

Indolent systemic mastocytosis

ISMs− :

Indolent systemic mastocytosis in the absence of skin lesions

ISMs+ :

Indolent systemic mastocytosis with skin lesions

MC:

Mast cells

MCAS:

Mast cell activation syndrome

MCL:

Mast cell leukemia

MH:

Methylhistamine

MIMA:

Methylimidazole acetic acid

MMAS:

Monoclonal mast cell activation syndrome

NSAIDs:

Nonsteroidal antiinflamatoy drugs

REMA:

Spanish Network on Mastocytosis

sBT:

Serum baseline tryptase

SM:

Systemic mastocytosis

VIT:

Venom immunotherapy

WAO:

World Allergy Organization

WDSM:

Well-differentiated systemic mastocytosis

WHO:

World Health Organization

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Conflict of Interest

Drs. Matito, Alvarez-Twose, Morgado, Sánchez-Muñoz, Orfao, and Escribano L all declare that they have no conflict of interest.

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Authors and Affiliations

  1. Instituto de Estudios de Mastocitosis de Castilla-La Mancha, Hospital Virgen del Valle, Toledo, Spain

    A. Matito, I. Alvarez-Twose, J. M. Morgado & L. Sánchez-Muñoz

  2. Centro de Investigación del Cáncer/IBMCC (USAL/CSIC) and IBSAL, Departamento de Medicina and Servicio General de Citometría, Campus Miguel de Unamuno, Universidad de Salamanca, 37007, Salamanca, Spain

    A. Orfao & L. Escribano

  3. Spanish Network on Mastocytosis (REMA), Toledo, Spain

    A. Matito, I. Alvarez-Twose, J. M. Morgado, L. Sánchez-Muñoz, A. Orfao & L. Escribano

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  1. A. Matito
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  2. I. Alvarez-Twose
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  3. J. M. Morgado
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  4. L. Sánchez-Muñoz
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  5. A. Orfao
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  6. L. Escribano
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Correspondence to L. Escribano.

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This article is part of the Topical Collection on Anaphylaxis and Drug Allergy

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Matito, A., Alvarez-Twose, I., Morgado, J.M. et al. Anaphylaxis as a Clinical Manifestation of Clonal Mast Cell Disorders. Curr Allergy Asthma Rep 14, 450 (2014). https://doi.org/10.1007/s11882-014-0450-8

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