In the Journal of Clinical Medicine, Suleiman and colleagues presented the case report of a patient with systemic mastocytosis (SM) who developed complications during an elective cardiac surgery. 

A 66-year-old woman who was diagnosed with SM around a decade ago was scheduled for an elective aortic valve replacement due to severe aortic valve stenosis. She also has a medical history of arterial hypertension, osteoporosis, and obesity. 

When the patient was initially diagnosed with SM, she presented with urticaria pigmentosa (cutaneous mastocytosis) with high tryptase levels (61.9 µg/L). A bone marrow biopsy was taken, revealing infiltration of approximately 10%. She was negative for c-KIT. 

Over the years, the patient’s manifestation of the disease was primarily dermatological (urticaria pigmentosa), which was well-controlled with 5 mg of cetirizine once daily and ultraviolet light therapy treatment during the fall and winter months. She was also provided with an emergency adrenaline autoinjector in the event that she experienced an anaphylactic episode. 

Read more about SM etiology 

Two hours before her aortic valve replacement surgery, her surgeons decided to give her an intravenous injection of 8 mcg of dexamethasone to avoid potential anaphylactic complications. The initial phase of the aortic valve replacement proceeded uneventfully, with the stenotic valve replaced with a valve prosthesis. She was then successfully weaned from cardiopulmonary bypass. However, during the reversal of heparin via the slow administration of protamine, the patient developed vasoplegia and critical hypotension. 

“Assuming that the causative factor was a histamine liberation due to an anaphylactic reaction attributable to the pre-existing systemic mastocytosis, epinephrine, antihistamines and corticosteroids were injected,” Suleiman et al wrote. 

The patient’s condition was successfully stabilized and she was transferred to the intensive care unit. She continued to be under moderate inotropic and vasopressor support. One day later, she was extubated and her catecholamines were stopped. 

However, she soon began to develop brown maculopapular patches and freckles, presumably due to the accumulation of mast cells in the skin. The presentation was consistent with cutaneous mastocytosis, which self-resolved prior to discharge.

During her hospital stay, she developed a bacterial infection which was successfully treated with antibiotics. She was discharged on postoperative day 13. The events during her surgery were described in her discharge letter to prevent further critical anaphylactic reactions in the patient. 

Complications in Surgery 

SM, characterized by the pathological buildup of mast cells in the body, often leads to multiorgan damage, with cardiovascular disease being particularly prominent. Roughly 1 in 4 cases of SM presents initially with cardiac arrest of unknown etiology. 

“Acute coronary syndrome and cardiac arrest are the most common cardiac complications of systemic mastocytosis that have been reported in the literature,” Battisha and colleagues wrote in Current Cardiology Reviews. 

Studies indicate that mast cells play a prominent role in the development of atherosclerosis. Patients diagnosed with SM have a higher rate of cardiovascular disease compared with the general population; this is irrespective of their cholesterol levels. In addition, epidemiological studies suggest that patients with SM also have a higher risk of developing cerebrovascular accidents. 

Severe cardiac disease, such as in the case of the patient described, may necessitate surgical correction. Because SM is a significant risk factor for dangerous anaphylactic reactions during surgery, careful preoperative planning is required on the part of both the surgeons and anesthesiologists involved.

“In systemic mastocytosis, the degranulation of the neoplastic mast cells that accumulate in various organs may be triggered by chemical, physical or psychological factors; infections; or medical procedures, including sedation, analgesia or multidrug pharmacotherapy,” Suleiman and colleagues explained. 

Thus, it is critical that surgeons are prepared in the event that an anaphylactic episode takes place intraoperatively. This means establishing prophylactic protocols, including preoperative preventive treatment and careful intraoperative monitoring. 

In the case of this patient, an anaphylactic episode occurred during the administration of protamine, most likely mediated by inflammatory mediators such as histamine. Hence, Suleiman and coauthors proposed that alternatives to the reversion of anticoagulation be used instead, such as prothrombin complex concentrate or fresh frozen plasma. 

Read more about SM treatment 

A Multidisciplinary Approach

This case study demonstrates the importance of considering all medical procedures needed to be undertaken in a patient with SM to be high risk and to take precautionary steps, including medical education. Multidisciplinary cooperation here is key. This is because the time window for the successful treatment of anaphylaxis associated with SM tends to be dangerously narrow, as was in the case of this patient. 

Because SM is an exceedingly rare condition, the medical literature on surgical complications in patients with SM is sparse. Nonetheless, there are comprehensive guidelines for the emergency management of anaphylaxis. In 2020, a panel of health professionals from 15 medical areas cooperated to issue 25 recommendations regarding the diagnosis, preparation, treatment, and postemergency care of anaphylaxis. Among their recommendations is that epinephrine be used as first-line treatment. 

In SM care, it is vital for there to be an increased awareness among healthcare professionals about the disease and that clinical research continues. High-quality, collaborative SM registries can also help physicians collect data on the best approaches to dealing with complications of the disease. 


Battisha A, Sawalha K, Madoukh B, et al. Acute myocardial infarction in systemic mastocytosis: case report with literature review on the role of inflammatory process in acute coronary syndromeCurr Cardiol Rev. 2020;16(4):333-337. doi:10.2174/1573403X16666200331123242

Suleiman MN, Brueckl V, Fechner J, et al. A practical approach to systemic mastocytosis complications in cardiac surgery: a case report and systematic review of the literatureJ Clin Med. 2023;12(3):1156. Published online February 1, 2023. doi:10.3390/jcm12031156

Li X, Ma Q, Yin J, et al. A clinical practice guideline for the emergency management of anaphylaxisFront Pharmacol. 2022;13:845689. Published online March 28, 2022. doi:10.3389/fphar.2022.845689