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Acute Non-Atherosclerotic ST-Segment Elevation Myocardial Infarction in an Adolescent with Concurrent Hemoglobin H-Constant Spring Disease and Polycythemia Vera.

Rattarittamrong E, Norasetthada L, Tantiworawit A, Chai-Adisaksopha C, Hantrakool S, Rattanathammethee T, Charoenkwan P - Hematol Rep (2015)

Bottom Line: Coronary artery angiography revealed an acute clot in the right coronary artery without atherosclerotic plaque.He was treated with plateletpheresis, hydroxyurea and antiplatelet agents.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.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Department of Internal Medicine.

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.

No MeSH data available.


Related in: MedlinePlus

A) Blood smear of the patient showing hypochromic microcytic red blood cells with anisocytosis and poikilocytosis compatible with thalassemia. Platelets were markedly increased with a variation in size and staining, including agranular platelets. B) Bone marrow aspirate revealing hypercellular marrow of 90%. There was panmyelosis or a proliferation of erythroid, granulocytic and megakaryocytic lineages with effective maturation. Megakaryocytes were increased in size with pleomorphism, and also nuclear lobulation. Significant platelet clumping was also seen.
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fig001: A) Blood smear of the patient showing hypochromic microcytic red blood cells with anisocytosis and poikilocytosis compatible with thalassemia. Platelets were markedly increased with a variation in size and staining, including agranular platelets. B) Bone marrow aspirate revealing hypercellular marrow of 90%. There was panmyelosis or a proliferation of erythroid, granulocytic and megakaryocytic lineages with effective maturation. Megakaryocytes were increased in size with pleomorphism, and also nuclear lobulation. Significant platelet clumping was also seen.

Mentions: A 17-year-old man was referred from a regional hospital to our institute for evaluation of his elevated platelet counts. He had an underlying disease of Hb H disease that required a splenectomy due to transfusion dependency when he was 5 years-old. The physical examination revealed no anemia, no jaundice, an enlarged liver to two centimeters below the right costal margin, and a splenectomy scar on his abdominal wall. The CBC showed a Hb of 11.5 g/dL, hematocrit (Hct) of 36.5%, corrected white blood cells (WBC) of 29,200/µL (neutrophils 66%, lymphocytes 16%, monocytes 8%, eosinophils 9%, basophils 1%), nucleated red cells (NRCs) 9/100 WBC, platelets 2,206,000/µL. Two months prior to his referral the patient’s medical record from the regional hospital showed a platelet count of 976,000/µL. His blood smear revealed marked thrombocytosis with variations in size and staining of the platelets, in addition to hypochromic microcytic red blood cells, NRCs and leukocytosis as shown in Figure 1A.


Acute Non-Atherosclerotic ST-Segment Elevation Myocardial Infarction in an Adolescent with Concurrent Hemoglobin H-Constant Spring Disease and Polycythemia Vera.

Rattarittamrong E, Norasetthada L, Tantiworawit A, Chai-Adisaksopha C, Hantrakool S, Rattanathammethee T, Charoenkwan P - Hematol Rep (2015)

A) Blood smear of the patient showing hypochromic microcytic red blood cells with anisocytosis and poikilocytosis compatible with thalassemia. Platelets were markedly increased with a variation in size and staining, including agranular platelets. B) Bone marrow aspirate revealing hypercellular marrow of 90%. There was panmyelosis or a proliferation of erythroid, granulocytic and megakaryocytic lineages with effective maturation. Megakaryocytes were increased in size with pleomorphism, and also nuclear lobulation. Significant platelet clumping was also seen.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4591500&req=5

fig001: A) Blood smear of the patient showing hypochromic microcytic red blood cells with anisocytosis and poikilocytosis compatible with thalassemia. Platelets were markedly increased with a variation in size and staining, including agranular platelets. B) Bone marrow aspirate revealing hypercellular marrow of 90%. There was panmyelosis or a proliferation of erythroid, granulocytic and megakaryocytic lineages with effective maturation. Megakaryocytes were increased in size with pleomorphism, and also nuclear lobulation. Significant platelet clumping was also seen.
Mentions: A 17-year-old man was referred from a regional hospital to our institute for evaluation of his elevated platelet counts. He had an underlying disease of Hb H disease that required a splenectomy due to transfusion dependency when he was 5 years-old. The physical examination revealed no anemia, no jaundice, an enlarged liver to two centimeters below the right costal margin, and a splenectomy scar on his abdominal wall. The CBC showed a Hb of 11.5 g/dL, hematocrit (Hct) of 36.5%, corrected white blood cells (WBC) of 29,200/µL (neutrophils 66%, lymphocytes 16%, monocytes 8%, eosinophils 9%, basophils 1%), nucleated red cells (NRCs) 9/100 WBC, platelets 2,206,000/µL. Two months prior to his referral the patient’s medical record from the regional hospital showed a platelet count of 976,000/µL. His blood smear revealed marked thrombocytosis with variations in size and staining of the platelets, in addition to hypochromic microcytic red blood cells, NRCs and leukocytosis as shown in Figure 1A.

Bottom Line: Coronary artery angiography revealed an acute clot in the right coronary artery without atherosclerotic plaque.He was treated with plateletpheresis, hydroxyurea and antiplatelet agents.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.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Department of Internal Medicine.

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.

No MeSH data available.


Related in: MedlinePlus