title: Management of Anesthesia in Adult and Pediatric Mastocytosis: A Study of the Spanish Network on Mastocytosis (REMA) Based on 726 Anesthetic Procedures.
abstract: BACKGROUND
  The role of anesthesia as an elicitor of mast cell (MC) mediator release symptoms in mastocytosis is poorly investigated.
  
  
  OBJECTIVE
  To determine the frequency and type of MC mediator release symptoms during anesthetic procedures in mastocytosis patients.
  
  
  METHODS
  Medical records were reviewed regarding the anesthetic techniques for 501 mastocytosis patients (459 adults and 42 children; 95 and 5% with systemic involvement, respectively) who were subjected to 676 and 50 anesthetic techniques, respectively. General, sedation, epidural, and local anesthetic techniques were used in 66 (10%), 67 (10%), 76 (11%), and 515 (76%) adult patients and in 24 (48%), 8 (16%), 2 (4%), and 25 (50%) pediatric patients.
  
  
  RESULTS
  The frequency of perioperative MC mediator-related symptoms and anaphylaxis was 2 and 0.4% in the adult series and 4 and 2% among children. In the adult series, this frequency was significantly higher in patients who previously presented with anaphylaxis (p = 0.03), underwent major surgeries (p < 0.001) and general anesthesia (p = 0.02), and were not given prophylactic antimediator therapy (PAT) 1 h before the anesthesia (H1/H2 antihistamines and benzodiacepines; p = 0.002).Hypersensitivity and/or allergy to the involved drugs and latex allergy were ruled out in all but one symptomatic case; when PAT was given and sedation was added, some cases later tolerated the same anesthetic drugs.
  
  
  CONCLUSIONS
  The frequency of perioperative anaphylaxis appears to be higher in mastocytosis patients than in the general population. Mastocytosis should not be a contraindication for anesthesia since PAT and adequate anesthetic management using the drugs with the safest profile appears to be effective in preventing/controlling MC mediator-associated symptoms.
authors.affiliation: Instituto de Estudios de Mastocitosis de Castilla-La Mancha, Hospital Virgen del Valle, Toledo, Spain.
authors.forename: Almudena
authors.initials: A
authors.lastname: Matito
authors.forename: José Mario
authors.initials: JM
authors.lastname: Morgado
authors.forename: Paula
authors.initials: P
authors.lastname: Sánchez-López
authors.forename: Iván
authors.initials: I
authors.lastname: Álvarez-Twose
authors.forename: Laura
authors.initials: L
authors.lastname: Sánchez-Muñoz
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.forename: Luis
authors.initials: L
authors.lastname: Escribano
mesh_terms: D000328:Adult; D000707:Anaphylaxis / Q000453:epidemiology / Q000276:immunology*; D000758:Anesthesia / Q000379:methods; D000777:Anesthetics / Q000276:immunology*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008407:Mast Cells / Q000276:immunology*; D034721:Mastocytosis, Systemic / Q000139:chemically induced / Q000276:immunology*; D019990:Perioperative Care; D012189:Retrospective Studies
chemical_list: D000777:Anesthetics