Chapter

Systemic mastocytosis

Tomás Ahern and Donal O’Shea

in Oxford Textbook of Endocrinology and Diabetes

Second edition

Published on behalf of Oxford University Press

ISBN: 9780199235292
Published online July 2011 | e-ISBN: 9780199608232 | DOI: http://dx.doi.org/10.1093/med/9780199235292.003.0671

Series: Oxford Textbooks

Systemic mastocytosis

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Mastocytosis is a heterogeneous group of rare disorders characterized by the abnormal growth and accumulation of mast cells in one or more organs. Mast cells are myeloid lineage cells that express the CD117 (KIT), CD45, and FcεRI cell surface markers. Patients with mastocytosis often present with abdominal cramps and diarrhoea and episodes of flushing, lightheadedness, and headache, which may prompt investigation for a neuroendocrine cause.

Cutaneous manifestations are common to all forms of mastocytosis. The majority of cases of mastocytosis are relatively benign, although some forms are associated with significant early death. The clinical course of mastocytosis is variable and can include shifting between the different forms.

Chapter.  3160 words.  Illustrated.

Subjects: Endocrinology and Diabetes

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