Acute Non-Atherosclerotic ST-Segment Elevation Myocardial Infarction in an Adolescent with Concurrent Hemoglobin H-Constant Spring Disease and Polycythemia Vera.
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.
Division of Hematology, Department of Internal Medicine.
Ekarat
E
Rattarittamrong
Division of Hematology, Department of Internal Medicine.
Lalita
L
Norasetthada
Division of Hematology, Department of Internal Medicine.
Adisak
A
Tantiworawit
Division of Hematology, Department of Internal Medicine.
Chatree
C
Chai-Adisaksopha
Division of Hematology, Department of Internal Medicine.
Sasinee
S
Hantrakool
Division of Hematology, Department of Internal Medicine.
Thanawat
T
Rattanathammethee
Division of Hematology and Oncology, Department of Pediatrics, Chiang Mai University , Thailand.
Pimlak
P
Charoenkwan
Polycythemia vera; hemoglobin H disease; hemoglobin H-Constant Spring disease; myocardial infarction; thalassemia