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Undiagnosed Systemic Mastocytosis Presenting as Postoperative Distributive Shock: A Case Report

Ripoll, Juan G. MD*; Ritter, Matthew J. MD*,†,‡; Comfere, Thomas B. MD*,†; Smith, Mark M. MD*,‡; Schaff, Hartzell V. MD§; Gilkey, George D. MD*,‡; Warner, Matthew A. MD*,†

doi: 10.1213/XAA.0000000000001093
Case Reports

Systemic mastocytosis (SM) is a rare clinical condition resulting from a clonal proliferation of abnormal mast cells. The clinical presentation may vary from mild cutaneous manifestations to aggressive systemic symptoms including intermittent episodes of anaphylaxis. We present a case of a 69-year-old male with abrupt and recurrent episodes of anaphylaxis with refractory distributive shock following cardiac surgery with cardiopulmonary bypass. Following a complex postoperative course, a bone marrow biopsy ultimately confirmed the diagnosis. Although rare, SM should be considered in the differential diagnosis of postoperative patients with unexplained and recurrent episodes of distributive shock.

From the *Department of Anesthesiology and Perioperative Medicine

Division of Critical Care Medicine

Division of Cardiovascular Anesthesia

§Division of Cardiothoracic Surgery, Mayo Clinic, Rochester, Minnesota.

Accepted for publication August 12, 2019.

Funding: Supported by Clinical and Translational Science Award (CTSA) grant No. KL2 TR002379 to Dr Warner from the National Center for Advancing Translational Science (NCATS). Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Institutes of Health (NIH).

The authors declare no conflicts of interest.

Address correspondence to Matthew A. Warner, MD, Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905. Address e-mail to

Copyright © 2019 International Anesthesia Research Society
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