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LVIV NATIONAL MEDICAL UNIVERSITY, UKRAINEFAMILY MEDICINE DEPARTMENT
CASE REPORT:ATYPICAL INITIAL MANIFESTATION OF
ESSENTIAL TROMBOCYTHEMIA (ET) WITH MYOCARDIAL INFARCTION
REPORTER: Assistant Professor PhD. SHATYNSKA-MYTSYK IRENE
CASE REPORTMale X., 75y/o, was admitted to the emergency room
with following primary diagnosis:
Acute coronary syndromeRight gonarthrosisRight femoral haematoma
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
• 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
• 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
Status localis• Massive painful
haematoma in the right femoral region
• Painful palpation of the right knee joint
Values 03.05.10 07.05.10 Normal range
Haemoglobin 128 130 120-140 g/L
RBC 3,7 3,8 3,7 – 4,7 х 1012
Plateletes 3420 х 109 3312 х 109 180 – 320 х 109
WBC 12 10 4-9 х 109
ESR 17 20 2 - 15 mm/hr
glucose 6,1 4,2 3,3-5,5 mmol/l
aspartate aminotransferase
0,62 0,38 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 5th day of hospital stayBiochemical workup showed stress glucose response, elevated AST
Coagulation tests
Values 06.05.2010 Normal range
Prothrombin time 16,0' 14,0' – 16,0' Prothrombin index 97% 85 -110% Lee-White coagulation time 12' 5'-10 ' Total fibrinogen 7,4 2 – 4 g/L
Delayed Bleeding time 12 min., elevated fibrinogen level 7,4 g/L
ADDITIONAL LABORATORY WORKUP
VALUE 08.05.10 NORMAL RANGE
Plasma trombopoetin ↑300 pg/mL 27–188 pg/mL
Other instrumental findings
ECG • Revealed sinus rhythm, HR 98/min, elevated ST segment and (–)
Т wave in І, ІІ standard and aVL, V5 – V6 leadsEchoCG 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
Chest X-ray
Within normal age and sex range
X-rays of the right knee
• Joint space narrowing• Subchondral sclerosis consistent with right knee
joint arthrosis
Bone marrow puncture
Increased cellularity of the BM due to megacariocytes, giant megacariocytes, minor fibrotic changes
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
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
DISCUSSION
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 polycythemia vera
• 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
Treatment protocol included:
1. Antiagregants: Aspirin 150 mg х 1 /daily
2. HYDROXYUREA 500 mg х 2 /daily
TREATMENT
Conclusions
In the early stages ET is usually asymptomatic, its usually a chance finding during blood workup
Frequently ET debuts with vascular thrombosisCoronary 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
APPRECIATE YOUR
ATTENTION !!!