Table 4

Perioperative management of patients with indolent systemic mastocytosis (adapted from published reviews52,56 )

PrincipleTimingSpecific measure
Multidisciplinary (anesthesia/hematology/allergy) preoperative evaluation 2 wk prior to surgery Review strategies for avoidance of potential/known provocative factors (eg, extremes of temperature, pressure, friction) during surgery 
Decide on anesthetic/analgesic medications: 
 1. Review prior drug exposure history and anesthesia records for information on drug tolerability 
 2. Lower-risk agents (preferred): inhalant anesthetic agents (fluranes), fentanyl, propofol, etomidate, ketamine, midazolam, cis-atracurium, pancuronium, vecuronium 
 3. Higher-risk agents (avoid): morphine, codeine, thiopental, succinylcholine, mivacurium, rocuronium, atracurium, NSAIDS 
Consider usefulness of skin/other allergy testing for selecting anesthetic and analgesic agents 
Maintaining baseline symptom control Morning of surgery and postoperatively Continue daily antimediator treatment regimen if feasible 
Premedications 8 to 12 h and 1 h prior to surgery Diphenhydramine, 25-50 mg PO/IV Ranitidine, 150 mg PO or 50 mg IV Montelukast, 10 mg PO (optional) Prednisone, 60 mg (for higher-risk cases) 
Anaphylaxis treatment Intra- and postoperatively Ensure preparedness to treat anaphylaxis at any time (eg, epinephrine, IV corticosteroids) 
Monitoring for breakthrough mast cell degranulation symptoms (including hypotension, bleeding) 24-72 h postoperatively Administer additional antihistamine agents and/or corticosteroids as needed 
PrincipleTimingSpecific measure
Multidisciplinary (anesthesia/hematology/allergy) preoperative evaluation 2 wk prior to surgery Review strategies for avoidance of potential/known provocative factors (eg, extremes of temperature, pressure, friction) during surgery 
Decide on anesthetic/analgesic medications: 
 1. Review prior drug exposure history and anesthesia records for information on drug tolerability 
 2. Lower-risk agents (preferred): inhalant anesthetic agents (fluranes), fentanyl, propofol, etomidate, ketamine, midazolam, cis-atracurium, pancuronium, vecuronium 
 3. Higher-risk agents (avoid): morphine, codeine, thiopental, succinylcholine, mivacurium, rocuronium, atracurium, NSAIDS 
Consider usefulness of skin/other allergy testing for selecting anesthetic and analgesic agents 
Maintaining baseline symptom control Morning of surgery and postoperatively Continue daily antimediator treatment regimen if feasible 
Premedications 8 to 12 h and 1 h prior to surgery Diphenhydramine, 25-50 mg PO/IV Ranitidine, 150 mg PO or 50 mg IV Montelukast, 10 mg PO (optional) Prednisone, 60 mg (for higher-risk cases) 
Anaphylaxis treatment Intra- and postoperatively Ensure preparedness to treat anaphylaxis at any time (eg, epinephrine, IV corticosteroids) 
Monitoring for breakthrough mast cell degranulation symptoms (including hypotension, bleeding) 24-72 h postoperatively Administer additional antihistamine agents and/or corticosteroids as needed 

NSAIDS, nonsteroidal anti-inflammatory drug; PO, by mouth; and IV intravenously.

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