Management

• No curative treatment; management consists of prevention of mediator effects and treatment of accompanying haematological disease where present.

• Avoid factors triggering acute mediator release: extremes of temperature, pressure, friction; aspirin, NSAIDs, opiates, alcohol, specific allergies.

• Treat acute mast cell mediator release.

• Anaphylaxis: epinephrine (adrenaline) 0.3mL of 1:1000 dilution (adult dose) every 10-15 minutes as needed.

262 • Refractory hypotension and shock: fluid resuscitation and epinephrine (adrenaline) IV bolus plus infusion of 1:10,000 dilution (up to 4-10mg/min); add inotropes if unresponsive.

• Commence H1 plus H2 receptor antagonists and steroids.

• Treat chronic mast cell mediator release: H1 plus H2 receptor antagonists: H1 antihistamines: diphenhydramine (25-50mg PO 4-6 hourly; 10-50mg IM/IV), hydroxyzine (25mg PO tid or qid; 25-100 mg IM/IV) or loratadine (non-sedating; 10mg PO od); H2 antihistamines: ranitidine (150mg PO bd; 50mg IV) or cimetidine (400-1600mg/day PO in divided doses; 300mg IV). Titrate doses for individual patient requirements; prednisolone 40-60 mg/day PO for malabsorption tailing; bis-phosphonates and radiotherapy for bone pain; PUVA for urticaria pigmentosa; a small number of patients gain symptomatic relief from interferon-a or cyclosporin-A for refractory symptoms.

• Treat any associated haematological disorder: generally achieve short partial remission at best; splenectomy may help pancytopenic patients. SCT should be considered in appropriate patients.

• Attempt to control organ infiltration by mast cells: daunorubicin plus cytarabine and CVP have been reported to produce partial responses.

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