Anaphylaxis as a Clinical Manifestation of Clonal Mast Cell Disorders

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...

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Veröffentlicht in:Current allergy and asthma reports 2014-08, Vol.14 (8), p.450-450, Article 450
Hauptverfasser: Matito, A., Alvarez-Twose, I., Morgado, J. M., Sánchez-Muñoz, L., Orfao, A., Escribano, L.
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container_end_page 450
container_issue 8
container_start_page 450
container_title Current allergy and asthma reports
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creator Matito, A.
Alvarez-Twose, I.
Morgado, J. M.
Sánchez-Muñoz, L.
Orfao, A.
Escribano, L.
description 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.
doi_str_mv 10.1007/s11882-014-0450-8
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subjects Allergology
Anaphylaxis - genetics
Anaphylaxis - immunology
Anaphylaxis - therapy
Anaphylaxis and Drug Allergy (Da Khan and M Castells
Humans
Mast Cells - immunology
Mast Cells - pathology
Mastocytosis - complications
Mastocytosis - immunology
Medicine
Medicine & Public Health
Section Editors
Topical Collection on Anaphylaxis and Drug Allergy
Treatment Outcome
title Anaphylaxis as a Clinical Manifestation of Clonal Mast Cell Disorders
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