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Anaphylaxis as a clinical manifestation of clonal mast cell disorders

AuthorsMatito, Almudena; Álvarez-Twose, Iván; Morgado, J. M.; Sánchez-Muñoz, Laura; Orfao, Alberto ; Escribano, Luis
KeywordsIndolent systemic mastocytosis in the absence of skin lesion
KIT mutation
Clonal mast cell disorders
Issue Date2014
CitationCurrent Allergy and Asthma Reports 14(8): 450 (2014)
AbstractClonal 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.
Identifiersdoi: 10.1007/s11882-014-0450-8
e-issn: 1534-6315
issn: 1529-7322
Appears in Collections:(IBMCC) Artículos
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