Thursday, 25 April 2013
Systemic mastocytosis
This week we talked about the management of anaphylaxis and a rare case of systemic mastocytosis. See our previous blog posts on anaphylaxis. Life saving key points:
- ABCs! Consider intubation early if there is stridor or airway edema
- 35% of intravascular volume can go extravascular in <10 minutes because of inappropriate vasodilation. IV fluids!
- The dose of epinephrine is 0.5 mg. Depending on the vial concentration, this is either 0.5mL of 1:1000 (1mg/ml) IM injection, or 5mL of 1:10 000 (0.1mg/mL) of a slow IV push
Mastocytosis is a disorder of excessive mast cell accumulation, arising from genetic mutations in the bone marrow. The clinical presentation may vary:
- Cutaneous: A variety of skin manifestations including nodules or a maculopapular rash. Darier's sign is urticaria and redness when the skin lesions are rubbed or scratched
- Systemic: Infiltration of mast cells into different organs can lead to end-organ effects, such as cytopenias
- Work-up for mastocytosis includes a serum tryptase level, bone marrow biopsy and skin biopsy if lesions are present
- Symptoms of mast cell degranulation, including recurrent anaphylaxis, can occur with both cutaneous and systemic forms. Identifying common triggers can help manage this condition, in addition to medications like H1/H2 blockers and anti-leukotrienes
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