Kounis Syndrome: A New Twist On an Old Disease

Abstract

Kounis syndrome is the concurrence of acute coronary syndromes with conditions associated with mast cell activation, such as allergies or hypersensitivity and anaphylactic or anaphylactoid insults that can involve other interrelated and interacting inflammatory cells behaving as a 'ball of thread'. It is caused by inflammatory mediators such as neutral proteases including tryptase and chymase, arachidonic acid products, histamine, platelet activating factor and a variety of cytokines and chemokines released during the activation process. Platelets with FCεRI and FCεRII receptors also participate in the above cascade. Vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells constitute the three reported variants of this syndrome. Kounis syndrome is a ubiquitus disease that represents a magnificent natural paradigm and nature's own experiment, in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture. Kounis syndrome can complicate anesthesia, vaccination, medical therapy and stent implantation and it seems to be associated with coronary allograft vasculopathy and takotsubo syndrome, it can often be confused with hypersensitivity myocarditis and can be the cause of unexplained sudden death. Kounis syndrome has revealed that the same mediators released from the same inflammatory cells are present in acute coronary events of nonallergic etiology. These cells are not only present in the culprit region before plaque erosion or rupture but they release their contents just before an actual coronary event. Therefore, does Kounis syndrome represent a magnificent natural paradigm and nature's own experiment in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture showing a novel way towards our effort to prevent acute coronary syndromes? Drugs, substances targeting the stem cell factor that is essential for mast cell development, proliferation, survival, adhesion and homing as well as monoclonal antibodies and natural molecules that protect mast cell surface and stabilize mast cell membrane could emerge as novel therapeutic ways capable to prevent acute coronary and acute cerebrovascular events.

Thrombotic responses to coronary stents, bioresorbable scaffolds and the Kounis hypersensitivity-associated acute thrombotic syndrome

“…The bare metal stents consist of a 316 L stainless steel platform containing nickel, chromium, titanium, manganese, and molybdenum (4). This metal platform, while can prevent the late luminal enlargement and further induce the beneficial vascular remodeling, fails to prevent restenosis that remains a crucial problem due to neointimal hyperplasia.…”

A Rarely Seen Type-I Kounis Syndrome Caused By Tetanus Vaccine

“…The primary cardiovascular effects of coronary vasoconstriction are plaque erosion, thrombocyte activation, dysrhythmia development, which are induced by various mechanisms and increments in the synthesis of tissue factors (8) . However, a patient suffering from Kounis syndrome, in addition to appropriate acute coronary syndrome management needs the determination of specific IgE antibodies, eosinophilia, serum histamine, and complement proteins for the identification of this disease (9) . Moreover, eosinophil, total IgE, and leukocyte levels were elevated in our patient.…”

Late Presentation of Pericarditis After Honey Bee Sting: Case report

“…Several cases of myocarditis and Kounis syndrome occurring after the honey bee sting have been reported. Type I Kounis syndrome includes patients with normal coronary arteries caused by acute release of inflammatory mediators during the allergic reaction (6). Epinephrine, dopamine and thromboxanes in honey bee venom can cause severe platelet aggregation, direct vasoconstriction and myocardial damage or dysfunction (7).…”

“…Epinephrine, dopamine and thromboxanes in honey bee venom can cause severe platelet aggregation, direct vasoconstriction and myocardial damage or dysfunction (7). Hypersensitivity myocarditis is diagnosed in the presence of eosinophils, atypical lymphocytes, and giant cells in myocardial biopsy, whereas biopsy in Kounis syndrome is typically normal (6). Late gadolinium enhancement in magnetic resonance imagination is highly specific for diagnosing of Kounis syndrome and myocarditis (8).…”