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 !!!