Systemic Mastocytosis Flashcards
(4 cards)
1
Q
What is it?
A
A neoplastic mast cell disorder that leads to systemic symptoms due to mast cell infiltration and degranulation
Classic sign is Dariers sign- urticaria after rubbing skin
2
Q
What are clinical features?
A
Dariers sign
Flushing- Episodic triggered by heat/alcohol/certain foods
GI symptoms e.g. abdo pain or diarrhoea
Pruritis
Possible hypotension or anaphylaxis episodes in severe disease
3
Q
What are the investigations?
A
Blood film- monocytosis
Serum tryptase elevated
Urinary histamine- increased excretion
Bone marrow biopsy is diagnostic
4
Q
What is the management?
A
1) Anti-histamines for flushing
2) Cytoreductive therapy in aggressive forms
