- Part 1 Principles of international endocrine practice
- Part 2 Pituitary and hypothalamic diseases
- Part 3 The thyroid
- Part 4 Parathyroid, calcium, and bone metabolism
- Part 5 The adrenal gland and endocrine hypertension
- Part 6 Neuroendocrine tumours and genetic disorders
- 6.1 Neuroendocrine tumours of the gastrointestinal tract: an appraisal of the past and perspectives for the future
- 6.2 Neuroendocrine tumour markers
- 6.3 Neuroendocrine (carcinoid) tumours and the carcinoid syndrome
- 6.4 Gastrinoma
- 6.5 Insulinoma and hypoglycaemia
- 6.6 Glucagonoma
- 6.7 VIPomas
- 6.8 Somatostatinoma
- 6.9 Imaging neuroendocrine tumours of the gastrointestinal tract
- 6.10 Systemic mastocytosis
- 6.11 Multiple endocrine neoplasia type 1
- 6.12 Multiple endocrine neoplasia type 2
- 6.13 von Hippel–Lindau disease and succinate dehydrogenase subunit (SDHB, SDHC, and SDHD) genes
- 6.14 Neurofibromatosis
- 6.15 Carney’s complex
- 6.16 Molecular and clinical characteristics of the McCune–Albright syndrome
- 6.17 Cowden’s syndrome
- Part 7 Growth and development during childhood
- Part 8 Female endocrinology and pregnancy
- Part 9 Male hypogonadism and infertility
- Part 10 Endocrinology of ageing and systemic disease
- Part 11 Endocrinology of cancer
- Part 12 Obesity, lipids, and metabolic disorders
- Part 13 Diabetes mellitus
(p. 940) Systemic mastocytosis
- Chapter:
- (p. 940) Systemic mastocytosis
- Author(s):
Tomás Ahern
and Donal O’Shea
- DOI:
- 10.1093/med/9780199235292.003.0671
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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- Part 1 Principles of international endocrine practice
- Part 2 Pituitary and hypothalamic diseases
- Part 3 The thyroid
- Part 4 Parathyroid, calcium, and bone metabolism
- Part 5 The adrenal gland and endocrine hypertension
- Part 6 Neuroendocrine tumours and genetic disorders
- 6.1 Neuroendocrine tumours of the gastrointestinal tract: an appraisal of the past and perspectives for the future
- 6.2 Neuroendocrine tumour markers
- 6.3 Neuroendocrine (carcinoid) tumours and the carcinoid syndrome
- 6.4 Gastrinoma
- 6.5 Insulinoma and hypoglycaemia
- 6.6 Glucagonoma
- 6.7 VIPomas
- 6.8 Somatostatinoma
- 6.9 Imaging neuroendocrine tumours of the gastrointestinal tract
- 6.10 Systemic mastocytosis
- 6.11 Multiple endocrine neoplasia type 1
- 6.12 Multiple endocrine neoplasia type 2
- 6.13 von Hippel–Lindau disease and succinate dehydrogenase subunit (SDHB, SDHC, and SDHD) genes
- 6.14 Neurofibromatosis
- 6.15 Carney’s complex
- 6.16 Molecular and clinical characteristics of the McCune–Albright syndrome
- 6.17 Cowden’s syndrome
- Part 7 Growth and development during childhood
- Part 8 Female endocrinology and pregnancy
- Part 9 Male hypogonadism and infertility
- Part 10 Endocrinology of ageing and systemic disease
- Part 11 Endocrinology of cancer
- Part 12 Obesity, lipids, and metabolic disorders
- Part 13 Diabetes mellitus