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Perioperative management of mastocytosis

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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.

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Data availability

Data is not available for outside review except for the FDA per institution IRB.

Abbreviations

ASM:

Aggressive systemic mastocytosis

CM:

Cutaneous mastocytosis

IgE:

Immunoglobulin E

ISM:

Indolent systemic mastocytosis

SM:

Systemic mastocytosis

SM-AHN:

Systemic mastocytosis with an associated hematologic neoplasm

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Acknowledgements

The Scientific Publications staff at Mayo Clinic provided editorial consultation and proofreading, administrative, and clerical support.

Funding

This work was supported by the Department of Anesthesiology and Perioperative Medicine, Mayo Clinic. The authors were responsible for data interpretation and preparation, review, and approval of the manuscript. The corresponding author had final responsibility for the decision to submit for publication.

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Correspondence to Toby N. Weingarten.

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Conflict of interest

T.N.W. reports financial support from Medtronic and Merck. J.H.B. receives royalty payments from licensing of the HMC-1 cell line.

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Lau, S., Sprung, J., Volcheck, G.W. et al. Perioperative management of mastocytosis. J Anesth 37, 741–748 (2023). https://doi.org/10.1007/s00540-023-03228-x

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