Blood Transfusion Components Inducing Severe Allergic Reactions: The First Case of Kounis Syndrome Induced by Platelet Transfusion

Abstract

Kounis syndrome is a multisystem and multidisciplinary disease affecting the circulatory system that can be manifested as spasm and thrombosis. It can occur as allergic, hypersensitivity, anaphylactic, or anaphylactoid reactions associated with the release of inflammatory mediators from mast cells and from other interrelated and interacting inflammatory cells, including macrophages and lymphocytes. A platelet subset with high- and low-affinity IgE surface receptors is also involved in this process. Whereas the heart, and particularly the coronary arteries, constitute the primary targets of inflammatory mediators, the mesenteric, cerebral, and peripheral arteries are also vulnerable. Kounis syndrome is caused by a variety of factors, including drugs, foods, environmental exposure, clinical conditions, stent implantation, and vaccines. We report a unique case of a 60-year-old male with a past medical history of allergy to human albumin, alcoholic cirrhosis, and esophageal varices, who was admitted due to multiple episodes of hematemesis. Due to low hemoglobin levels, he was transfused with 3 units of red blood cells and fresh frozen plasma without any adverse reactions. On the third day of hospitalization, severe thrombocytopenia was observed and transfusion of platelets was initiated. Immediately following platelet infusion, the patient developed chest discomfort, skin signs of severe allergic reaction, and hemodynamic instability. The electrocardiogram revealed ST segment elevation in the inferior leads. Given the strong suspicion of Kounis syndrome/allergic coronary spasm, the patient was treated with anti-allergic treatment only, without any anti-platelet therapy. The clinical status of the patient gradually improved and the electrocardiographic changes reverted to normal. Based on these findings, Kounis hypersensitivity-associated acute coronary syndrome, specifically, type I Kounis syndrome, was diagnosed. Although platelet transfusion can be a life-saving therapy, each blood transfusion carries a substantial risk of adverse reactions. The aims of this report are to expand the existing knowledge of patient responses to blood transfusion and provide information on the incidence of various severe transfusion reactions to all blood components and especially to platelets. To the best of our knowledge, Kounis syndrome induced by platelet transfusionhas never been previously reported. Hypersensitivity to platelet external membrane glycoproteins in an atopic patient seems to be the possible etiology. Despite that Kounis syndrome remains an under-diagnosed clinical entity in everyday practice, it should always be considered in the differential diagnosis of acute coronary syndromes.

Keywords: Kounis syndrome; allergy; anaphylaxis; blood components; blood transfusion; platelets; transfusion.

Figure 1

Kounis syndrome. Current classification in 3 subtypes based on allergic pathogenic mechanisms and ensuing effects. Type 1 usually occurs in normal or near-normal coronary arteries, largely due to histamine degranulation from mast cells, catecholamine storm, endothelin release, and other cytokines (MINOCA syndrome). Type 2 is usually seen in patients with quiescent pre-existing coronary artery disease (CAD) and may result in acute coronary syndrome/myocardial infarction. Type 3 affects patients with coronary artery stenting, with subsequent acute thrombosis or restenosis. Ag, antigen; IgE, immunoglobulin E; IL6, interleukin 6; MINOCA, myocardial infarction with non-obstructive coronary arteries; PAF, platelet activation factor; PF4, platelet factor 4.

Figure 2

The emergency electrocardiogram, performed under anaphylactic shock conditions, showing ST segment elevation in the inferior leads, as well as ST segment depression in the anterior leads with first-degree atrioventricular block.

Figure 3

Following administration of anti-allergic treatment, a new electrocardiogram showed sinus tachycardia with complete resolution of ST-segment elevation and first-degree atrioventricular block.