Systemic Mastocytosis: Case Report and Narrative Review with Practical Recommendations to The Anesthesiologists

Mastocytosis is a rare disease with increased clonal mast cells prone to activation and degranulation that has a substantial impact on choice of anesthetic agents and perioperative management. Patients with mastocytosis have up to a six-fold increased risk for anaphylaxis and an increased risk for severe perioperative immediate hypersensitivity reactions (IHR). The prevalence for allergic (immunoglobulin E- mediated) IHR is similar to the general population even if concurrent allergic diseases, which are generally frequent, are also possible. Non-immunological mechanisms involving mast cell receptors are mainly responsible for mast cell degranulation. Risk factors for perioperative IHR in adult patients with mastocytosis are previous anaphylaxis, major surgery, general anesthesia, and lack of adequate premedication. While unspecific triggers (physical, thermal, psychological) are well-accepted cofactors in perioperative IHR, a definitive classification of safe or unsafe drugs remains controversial. Management of patients with mastocytosis requires multidisciplinary and specific preparation for perioperative management, adequate premedication, avoidance of triggering factors, and careful choice of anesthetic drugs.

Keywords:Systemic mastocytosis; General anesthesia; Perioperative management; Neuromuscular blocking drugs; Case report; Narrative review

Abbreviations:ASA: American Society of Anesthesiology; aVWS: acquired von Willebrand syndrome; HES: hydroxyethyl starch; IgE: immunoglobulin E; IHR: immediate hypersensitivity reaction; LA: local anesthetic; MPCM: maculopapular cutaneous mastocytosis; MRGPRX2: Masrelated G-protein coupled receptor member X2; NMBA: neuromuscular blocking agents; NSAIDs: non-steroidal anti-inflammatory drugs; VWF: von Willebrand factor