Mastocytosis

Mastocytosis is a disorder of abnormal mast cell proliferation, with clinical features that include flushing, pruritus, abdominal pain, diarrhea, hypotension, syncope, and musculoskeletal pain. These features are the result of mast cell mediator release and infiltration into target organs. Patients...

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Bibliographic Details
Published inImmunology and allergy clinics of North America Vol. 34; no. 1; p. 181
Main Authors Carter, Melody C, Metcalfe, Dean D, Komarow, Hirsh D
Format Journal Article
LanguageEnglish
Published United States 01.02.2014
Subjects
Disease Progression
Humans
Mastocytosis - diagnosis
Mastocytosis - etiology
Mastocytosis - therapy
Prognosis
Treatment Outcome
Bone marrow
Mastocytosis
Mast cell
Skin
Urticaria
KIT
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Summary:Mastocytosis is a disorder of abnormal mast cell proliferation, with clinical features that include flushing, pruritus, abdominal pain, diarrhea, hypotension, syncope, and musculoskeletal pain. These features are the result of mast cell mediator release and infiltration into target organs. Patients of all ages may be affected, although in children, manifestations primarily involve the skin. Most patients with systemic disease have a somatically acquired activating mutation in the KIT oncogene. This article discusses the causes and pathogenesis of mastocytosis, with an overview of the clinical features and the approach to diagnosis, evaluation, and therapy in adults and pediatric patients.
ISSN:1557-8607
DOI:10.1016/j.iac.2013.09.001