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Acute Non-Atherosclerotic ST-Segment Elevation Myocardial Infarction in an Adolescent with Concurrent Hemoglobin
Ekarat Rattarittamrong1, Lalita Norasetthada1, Adisak Tantiworawit1
1Division of Hematology, Department of Internal Medicine.
Insights
Polycythemia vera (PV) and hemoglobin H-Constant Spring disease can increase thrombosis risk. Early diagnosis and treatment of PV in thalassemia patients with high platelets are crucial for preventing heart attacks.
Area of Science:
- Hematology
- Cardiology
- Genetics
Background:
- Thrombosis is a significant complication in both polycythemia vera (PV) and thalassemia.
- JAK2 V617F mutation is a common driver in PV, associated with increased thrombotic risk.
Observation:
- A 17-year-old male with post-splenectomized hemoglobin H-Constant Spring disease and JAK2 V617F-positive PV presented with extreme thrombocytosis (platelets > 1,000,000/µL).
- Three months later, he experienced an ST-segment elevation myocardial infarction (STEMI) due to an acute coronary artery clot without atherosclerotic plaque.
Findings:
- The patient was treated with plateletpheresis, hydroxyurea, and antiplatelet agents.
- Treatment led to a decrease in platelet count and improvement in symptoms.
Implications:
- This case highlights the critical need for early diagnosis and management of PV in patients with underlying thalassemia and marked thrombocytosis.
- Awareness of heightened thrombotic risk in such patients is essential for preventing severe cardiovascular events like STEMI.
Abstract:
Thrombosis is a major complication of polycythemia vera (PV) and also a well-known complication of thalassemia. We reported a case of non-atherosclerotic ST-segment elevation myocardial infarction (STEMI) in a 17-year-old man with concurrent post-splenectomized hemoglobin H-Constant Spring disease and JAK2 V617F mutation-positive PV. The patient initially presented with extreme thrombocytosis (platelet counts greater than 1,000,000/µL) and three months later developed an acute STEMI. Coronary artery angiography revealed an acute clot in the right coronary artery without atherosclerotic plaque. He was treated with plateletpheresis, hydroxyurea and antiplatelet agents. The platelet count decreased and his symptoms improved. This case represents the importance of early diagnosis, awareness of the increased risk for thrombotic complications, and early treatment of PV in patients who have underlying thalassemia with marked thrombocytosis.
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