Introduction: Prinzmetal anginais a special type ofacute coronary syndrome ST+ wich correspond to a transient occlusion of a coronary vessel secondary to spasm. This type of acute coronary syndrome is very rare and is characterized by the presence of signs of myocardial ischemia on electrocardiogram but coronary angiography and coroscaner are frequently normals. Its management in hemophilia patients is difficult because of the use of anticoagulant and antiplatelet drugs wich increase bleeding risk. We report the case of a major hemophilia A patient which presented Prinzmetal angina.

Observation: It is a 64 years old patient, hemophilia A major, chronic smoking (40 pack-year), not diabetic, not hypertensive, which had a retro sternal constrictive pain radiating to the shoulders. At admission he was consciousness. No breath in cardiac auscultation. The electrocardiography showed a heart rate at 61bpm, the axis of the heart was normal. ST-segment elevation was noticed in DIII and AVF: ischemia in the cardiac lower area. Echocardiography was normal. The coroscaner was normal. The troponin I level was at 0.03 µg/L (Normal: 0-0.1µg/L). This patient had a variant Prinzmetal angina. Treatement: Diltiazem Hydrochloride 60 mg 1 tablet / 8 hours. Acetylsalicylic acid 160 mg IV and Clopidogrel 300 mg IV the first day; relay with acetylsalicylic acid 100 mg and clopidogrel 75 mg per day. Transfusion of factor VIII at the dose of 40UI / Kg. Simvastatin 20 mg 1 tablet per day. Perindopril 5 mg 1 tablet the day. The evolution was favorable.

Discussion and conclusion: Coronary syndromes are not frequent in morocco hemophilia patients. Their management is complex and involves the presence of an hematologist.

Disclosures

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.

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