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Systemic mastocytosis 

Systemic mastocytosis
Chapter:
Systemic mastocytosis
Author(s):

Tomás Ahern

and Donal O’Shea

DOI:
10.1093/med/9780199235292.003.0671
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date: 17 May 2022

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.

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