LVIV NATIONAL MEDICAL UNIVERSITY, UKRAINE FAMILY MEDICINE DEPARTMENT

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 tothe 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. PATIENT’S HISTORY • 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

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

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

7. INITIAL LABORATORY EVALUATION: Blood work revealed significantly elevated level of plateletes (more than 11 times higher than normal range), leukocytosis, ESR increased on the 5th day of hospital stay Biochemical workup showed stress glucose response, elevated AST

8. Coagulation tests Delayed Bleeding time 12 min., elevated fibrinogen level 7,4 g/L

9. ADDITIONAL LABORATORY WORKUP

10. Other instrumental findings ECG • Revealed sinus rhythm, HR 98/min, elevated ST segment and (–) Т wavein І, ІІ standardand aVL, V5 – V6 leads EchoCG showedimpairment 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. Joint space narrowing Subchondral sclerosis consistent with right knee joint arthrosis X-rays of the right knee

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

14. CLINICAL DIAGNOSIS: in accordance to the patient’s history, symptoms and the subsequent lab. work up 

15. DISCUSSION 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 development

16. Diagnostic criteria of ET by Polycythemia Vera Study Group, 2005 • The diagnosis requires the presence of a persistent thrombocytosis of greater than 600 × 103/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 × 103/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 polycythemiavera • hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores • B4. No evidence of chronic myeloid leukemia • But the Philadelphia chromosome may be present in up to 10% of cases. • B5. No evidence of myelofibrosis • B6. No evidence of a myelodysplastic syndrome

17. TREATMENT • Treatment protocol included: • Antiagregants: Aspirin 150 mg х 1 /daily • HYDROXYUREA500 mg х 2 /daily

18. Conclusions

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