Essential thrombocythemia presenting as acute coronary syndrome: case reports and literature review
Nanqing Xiong1, Wen Gao1, Junjie Pan1
1Department of Cardiology, Huashan Hospital, Fudan University, 12 Wulumuqizhong Road, Shanghai, China.
Insights
Essential thrombocythemia (ET) can present as acute coronary syndrome (ACS), even with mild thrombocytosis. Early diagnosis and intensive treatment combining anti-platelet therapy, cytoreduction, and intervention improve outcomes for these high-risk patients.
Area of Science:
- Hematology
- Cardiology
- Oncology
Background:
- Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by excessive platelet production.
- Systemic thrombosis is a major complication of ET, but its presentation as acute coronary syndrome (ACS) is rare.
- Patients with ACS lacking typical risk factors may have undiagnosed underlying ET.
Observation:
- Three cases of ACS were reported in patients without common coronary artery disease risk factors and with only mild atherosclerotic stenosis.
- Elevated platelet levels and specific angiographic findings prompted further investigation for ET.
- Diagnosis was confirmed via bone marrow morphology and genetic testing.
Findings:
- Despite mild thrombocytosis, intensive treatment including dual anti-platelet therapy, cytoreduction, and early coronary intervention yielded positive outcomes.
- Patients were stratified as high-risk for ET.
- Complete response was defined as a platelet count below 400 × 10^9/L.
Implications:
- Cardiologists should consider ET in ACS patients with thrombocytosis, lack of risk factors, and non-typical coronary artery occlusion.
- Combined treatment strategies are beneficial for managing ET presenting as ACS.
- This highlights the importance of a multidisciplinary approach in diagnosing and treating complex thrombotic events.
Abstract:
For the patients with essential thrombocythemia (ET), systemic thrombosis presents as one of the most dangerous complications. It's been widely accepted that acute coronary syndrome (ACS) is a kind of thrombotic diseases. However, there are very few case reports about ET first presenting as ACS. For some patients diagnosed as ACS, but without markedly elevated platelet, underlying ET was missed. And there are some controversies in the principles and target of treatment in those patients. We reported three cases of ACS, in which the patients who did not have common risk factors for coronary artery diseases and presented only mild atherosclerotic stenosis during coronary angiography, one of which had recurrent coronary artery thrombosis. Noticing their elevated blood platelet level and characteristics in angiography, diagnosis of ET was made according to bone marrow morphology and genetic tests. Although they had only mild thrombocytosis, we applied intensive treatment with dual anti-platelet therapy combined with cytoreduction in addition to early coronary intervention, having satisfying outcomes. During the diagnosis and treatment of ACS, if patients present thrombocytosis, but lack common coronary disease risk factors and thrombotic coronary artery occlusion, cardiologists should search for possible ET as an underlying cause of thrombotic coronary event. All those patients were high-risk according to ET risk stratification. Treatment of cytoreduction in combination with anti-thrombosis therapy and revascularization are beneficial. Treatment aims at the target of complete response with platelet count below 400 × 109/L.
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