Anaphylaxis As a Clinical Manifestation of Clonal Mast Cell Disorders

Overview

Journal Curr Allergy Asthma Rep

Specialties Allergy & Immunology
Pulmonary Medicine

Date 2014 Jun 21

PMID 24947681

Citations 11

Authors

A Matito

I Alvarez-Twose

J M Morgado

L Sanchez-Munoz

A Orfao

L Escribano

Affiliations

Soon will be listed here.

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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