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2017 ; 8
(ä): 792
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Insights in Anaphylaxis and Clonal Mast Cell Disorders
#MMPMID28740494
González-de-Olano D
; Álvarez-Twose I
Front Immunol
2017[]; 8
(ä): 792
PMID28740494
show ga
The prevalence of anaphylaxis among patients with clonal mast cell disorders
(MCD) is clearly higher comparing to the general population. Due to a lower
frequency of symptoms outside of acute episodes, clonal MCD in the absence of
skin lesions might sometimes be difficult to identify which may lead to
underdiagnosis, and anaphylaxis is commonly the presenting symptom in these
patients. Although the release of mast cell (MC) mediators upon MC activation
might present with a wide variety of symptoms, particular clinical features
typically characterize MC mediator release episodes in patients with clonal MCD
without skin involvement. Final diagnosis requires a bone marrow study, and it is
recommended that this should be done in reference centers. In this article, we
address the main triggers for anaphylaxis, risk factors, clinical presentation,
diagnosis, and management of patients with MC activation syndromes (MCASs), with
special emphasis on clonal MCAS [systemic mastocytosis and mono(clonal) MC
activations syndromes].