1. LVIV NATIONAL MEDICAL UNIVERSITY, UKRAINE FAMILY MEDICINE DEPARTMENT CASE REPORT: ATYPICAL INITIAL MANIFESTATION OF ESSENTIAL TROMBOCYTHEMIA (ET) WITH MYOCARDIAL INFARCTION REPORTER: Assistant Professor PhD. SHATYNSKA-MYTSYK IRENE

2. CASE REPORT Male X., 75y/o, was admitted to the emergency room with following primary diagnosis: Acute coronary syndrome Right gonarthrosis Right femoral haematoma

3. COMPLAINTS on: sudden onset of retrosternal chest pain lasting > 30 min. that wasn’t relieved by intake of 2 doses of nitroglycerine left arm numbness shortness of breath (dyspnea) cold sweat (diaphoresis) anxiety, weakness

4. Detailed questioning revealed that recently patient felt weakness in the lower limbs suffered from intermittent headaches 2 weeks prior hospitalization had minor right hip trauma PATIENT’S HISTORY

5. Patient appeared pale and diaphoretic Slightly hypotensive 100/60 mm Hg Accelerated HR 98/min Enlarged liver +5cm other findings occurred inconclusive PHYSICAL EXAMINATION IN THE EMERGENCY WARD

6. Status localis Massive painful haematoma in the right femoral region Painful palpation of the right knee joint

7. Values03.05.1007.05.10Normal range Haemoglobin128130120-140 g/L RBC3,73,83,7 – 4,7 х 10 12 Plateletes3420 х 10 9 3312 х 10 9 180 – 320 х 10 9 WBC 12104-9 х 10 9 ESR17202 - 15 mm/hr glucose6,14,23,3-5,5 mmol/l aspartate aminotransferase 0,620,38 0,1-0,42 mmol/l INITIAL LABORATORY EVALUATION: Blood work revealed significantly elevated level of plateletes (more than 11 times higher than normal range), leukocytosis, ESR increased on the 5 th day of hospital stay Biochemical workup showed stress glucose response, elevated AST

8. Coagulation tests Values06.05.2010 Normal range Prothrombin time16,0'14,0' – 16,0' Prothrombin index97%85 -110% Lee-White coagulation time12'5'-10 ' Total fibrinogen7,42 – 4 g/L Delayed Bleeding time 12 min., elevated fibrinogen level 7,4 g/L

9. ADDITIONAL LABORATORY WORKUP VALUE08.05.10NORMAL RANGE Plasma trombopoetin↑300 pg/mL 27–188 pg/mL

10. Other instrumental findings ECG Revealed sinus rhythm, HR 98/min, elevated ST segment and (–) Т wave in І, ІІ standard and aVL, V5 – V6 leads EchoCG showed impairment of segmental contractility (hypokinesis) of anterior and lateral wall of LV, with inconclusive chamber size and volumetric parameters, preserved EF 53% Abdominal US enlarged liver with slightly increased echogenicity spleen within normal range

11. Chest X-ray Within normal age and sex range

12. X-rays of the right knee Joint space narrowing Subchondral sclerosis consistent with right knee joint arthrosis

13. Bone marrow puncture Increased cellularity of the BM due to megacariocytes, giant megacariocytes, minor fibrotic changes

14. Acute anterior lateral STEMI Primary essential trombocythemia Right gonarthrosis CLINICAL DIAGNOSIS: in accordance to the patient’s history, symptoms and the subsequent lab. work up 

15. Essential thrombocytosis (ET, also known as Primary thrombocytosis (PT) is a rare chronic blood cancer characterized by the overproduction of platelets by megakaryocytes in the BM, accompanied by increased blood viscosity resulting in thrombosis developmentoverproductionplateletsmegakaryocytes DISCUSSION

16. Diagnostic criteria of ET by Polycythemia Vera Study Group, 2005 The diagnosis requires the presence of a persistent thrombocytosis of greater than 600 × 10 3 /µL in the absence of an alternative cause The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6. A1. Platelet count > 600 × 10 3 /µL for at least 2 months A2. Acquired V617F JAK2 mutation present B1. No cause for a reactive thrombocytosis normal inflammatory indices B2. No evidence of iron deficiency B3. No evidence of polycythemia verapolycythemia vera hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores B4. No evidence of chronic myeloid leukemiachronic myeloid leukemia But the Philadelphia chromosome may be present in up to 10% of cases.Philadelphia chromosome B5. No evidence of myelofibrosismyelofibrosis B6. No evidence of a myelodysplastic syndrome

17. Treatment protocol included: 1.Antiagregants: Aspirin 150 mg х 1 /daily 2.HYDROXYUREA 500 mg х 2 /daily TREATMENT

18. Conclusions In the early stages ET is usually asymptomatic, its usually a chance finding during blood workup Frequently ET debuts with vascular thrombosis Coronary artery thrombosis gives a clinical presentation of acute myocardial infarction Presence of ET worsens the course of coronary artery disease, hypertension, obliterating vascular diseases, cerebral atherosclerosis

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