Perioperative Management of Mastocytosis

Preoperative Evaluation

The American College of Allergy, Asthma, and Immunology recommends that patients with mastocytosis or suspected mast cell disorders should have a baseline tryptase level measured preoperatively to reflect mast cell burden and help interpret subsequent acute elevations 2, 3
A baseline tryptase level does not necessarily predict perioperative adverse events, but its knowledge is essential for differential diagnosis if a reaction occurs 2
Approximately 5-7% of the population has elevated baseline tryptase without relation to anaphylaxis, including patients with hereditary alpha-tryptasemia 4

The National Comprehensive Cancer Network recommends premedication for most procedures in patients with systemic mastocytosis, as surgery, endoscopy, and other invasive procedures can induce mast cell activation and anaphylaxis 5
The use of benzodiazepines, antihistamines (H1 and H2 blockers), and corticosteroids likely helps reduce the frequency and/or severity of mast cell activation symptoms 1
Antihistamines H1 (such as cetirizine, diphenhydramine, or hydroxyzine) combined with antihistamines H2 (ranitidine or famotidine) are particularly effective 6
Corticosteroids: hydrocortisone 200 mg IV (adult dose) as part of the prophylactic regimen 7

The American Society of Anesthesiologists recommends that all patients should have immediate access to epinephrine, with two auto-injectors available for anaphylaxis management 5
The risk of anaphylaxis in the perioperative period is higher in patients with systemic mastocytosis compared to the general population 1

The American Heart Association recommends immediate treatment with epinephrine IM 0.01 mg/kg (1:1000 concentration) in the anterolateral thigh, maximum dose 0.5 mg in adults and 0.3 mg in children, as first-line treatment without absolute contraindications 9
Administer oxygen at 100% immediately 9
Establish large-caliber IV access and administer rapid boluses of 0.9% saline or Ringer's lactate solution 7, 9

The American College of Allergy, Asthma, and Immunology recommends that all patients with suspected anaphylactic reactions associated with anesthesia should be fully investigated 7
In case of anaphylaxis or other mast cell activation events, initiate a complete allergologic study, including skin tests or detection of specific IgE antibodies to identify IgE-mediated hypersensitivity to drugs 1
Refer to a specialized center in Allergy or Immunology with appropriate experience 7