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Review
. 2023 Oct;37(5):741-748.
doi: 10.1007/s00540-023-03228-x. Epub 2023 Jul 19.

Perioperative management of mastocytosis

Sirimas Lau  1 , Juraj Sprung  1 , Gerald W Volcheck  2 , Joseph H Butterfield  2 , Rohit D Divekar  2 , Toby N Weingarten  3   4
Affiliations
Review

Perioperative management of mastocytosis

Sirimas Lau et al. J Anesth. 2023 Oct.

Abstract

Purpose: Patients with mastocytosis have an increased risk of anaphylaxis during surgical procedures with general anesthesia. Therefore, we reviewed the anesthesia course of a large cohort of patients with mastocytosis.

Methods: We retrospectively reviewed adult and pediatric patients with mastocytosis who underwent surgical procedures with general anesthesia at Mayo Clinic from January 1, 2000, through June 30, 2021. We also included any procedures with general anesthesia that occurred during the 3-year period preceding mastocytosis diagnosis and designated the patients who underwent these procedures as having an unknown diagnosis at the time of their surgical procedure. We analyzed whether patients received chronic antimediator treatment for mastocytosis and/or prophylactic medications before the procedures. We also determined whether medications indicative of mastocytosis-related adverse events were intraoperatively administered.

Results: We identified 113 patients who underwent 219 procedures during the study period; 25 procedures were performed before mastocytosis diagnosis. Of 194 procedures in patients with known mastocytosis, patients received chronic antimediator therapy and/or perioperative prophylactic medications for 178 (91.8%) procedures. Among these procedures, 10 were potentially complicated by mast cell activation, which was inferred from administration of inhaled albuterol (n = 3) or intravenous diphenhydramine (n = 8). In addition, there was only one case of intraoperative anaphylaxis which occurred in a patient who underwent anesthesia before mastocytosis diagnosis and therefore did not receive prophylaxis.

Conclusion: Intraoperative anaphylaxis can be the first presenting sign of mastocytosis. Patients with mastocytosis who received chronic antimediator therapy and/or preoperative prophylactic medications had an uneventful surgical course.

Keywords: Anaphylaxis; Cutaneous mastocytosis; General anesthesia; Prophylaxis; Systemic mastocytosis.

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References

    1. Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin North Am. 2014;34(2):283–95. https://doi.org/10.1016/j.iac.2014.01.003 . - DOI - PubMed
    1. Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nunez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603–25. https://doi.org/10.1016/s0145-2126(01)00038-8 . - DOI - PubMed
    1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391–405. https://doi.org/10.1182/blood-2016-03-643544 . - DOI - PubMed
    1. Castells MC, Akin C. Mastocytosis (cutaneous and systemic) in children: epidemiology, clinical manifestations, evaluation, and diagnosis. 2022. https://www.uptodate.com/contents/mastocytosis-cutaneous-and-systemic-in... . Accessed 31 Jan 2023.
    1. Romantowski J, Gorska A, Niedoszytko M, Gulen T, Gruchala-Niedoszytko M, Nedoszytko B, Lange M, Brockow K, Arock M, Akin C, Valent P. A challenge for allergologist: application of allergy diagnostic methods in mast cell disorders. Int J Mol Sci. 2021;22(3):1454. https://doi.org/10.3390/ijms22031454 . - DOI - PubMed - PMC

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