期刊
CURRENT ALLERGY AND ASTHMA REPORTS
卷 14, 期 8, 页码 -出版社
CURRENT MEDICINE GROUP
DOI: 10.1007/s11882-014-0450-8
关键词
Anaphylaxis; Clonal mast cell disorders; Indolent systemic mastocytosis in the absence of skin lesion; KIT mutation
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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