Acute non-atherosclerotic ST-segment elevation myocardial infarction in an adolescent with concurrent hemoglobin H-Constant Spring disease and polycythemia vera

  • Ekarat Rattarittamrong | ekarat_r@hotmail.com Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Lalita Norasetthada Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Adisak Tantiworawit Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Chatree Chai-Adisaksopha Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Sasinee Hantrakool Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Thanawat Rattanathammethee Division of Hematology, Department of Internal Medicine, Department of Pediatrics, Chiang Mai University, Thailand.
  • Pimlak Charoenkwan Division of Hematology and Oncology, Department of Pediatrics, Chiang Mai University, Thailand.

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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Published
2015-09-23
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Section
Case Reports
Keywords:
Polycythemia vera, hemoglobin H disease, hemoglobin H-Constant Spring disease, thalassemia, myocardial infarction
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How to Cite
Rattarittamrong, E., Norasetthada, L., Tantiworawit, A., Chai-Adisaksopha, C., Hantrakool, S., Rattanathammethee, T., & Charoenkwan, P. (2015). Acute non-atherosclerotic ST-segment elevation myocardial infarction in an adolescent with concurrent hemoglobin H-Constant Spring disease and polycythemia vera. Hematology Reports, 7(3). https://doi.org/10.4081/hr.2015.5941