Young Male Presenting with Acute Coronary Syndrome and Borderline
Platelets Revealed Essential Thrombocythemia case report
Mohammad K Alzyod1, Ahmad s. matarneh 1, Mohamed A. Yassin2
1 Department of Medicine, Hamad Medical corporation, Doha, Qatar
2 Department of Medical Oncology, Hematology section, National center
for cancer and Research, Hamad Medical Corporation, Doha, Qatar
Corresponding Author
Ahmad matarneh
Department of Medicine, Hamad Medical corporation, Doha, Qatar
Rayan street
P.O box 3050
Ahmadmatarneh99@gmail.com
Tel: +97455957396
Short Title:
Acute Coronary Syndrome and Essential Thrombocythemia
Key words
Essential thrombocythemia, Acute coronary syndrome, Myeloproliferative
neoplasms, Cardiovascular risks, Myocardial infarction, Case report
Abstract Background:
Essential thrombocythemia is myeloproliferative neoplasm, with the risk
of progression to other cancers. Borderline platelet can be over seen
and passed as normal or upper limit of normal. So complications can
happen before diagnosis and treatment.
Case presentation:
This study reports a gentleman who is 32 years old; presented with
typical chest pain, and diagnosed with acute coronary syndrome, coronary
angiography showed 90% obstruction in the left anterior descending
artery, percutaneous coronary intervention was done for him. Due to
absence of risk factors, he was referred to hematology after 4 years of
presentation and diagnosed with
Conclusion:
This study will highlight the importance of screening for
myeloproliferative neoplasm in young with borderline platelet.
We also emphasize that early diagnosis and considering essential
thrombocythemia and starting treatment as soon as possible can affect the
outcome and prevent complications.
Introduction
Based on the British Journal of Haematology, Myeloproliferative
neoplasms are a group of hematopoietic myeloid neoplasms, classically
described as Philadelphia positive myeloproliferative neoplasm; chronic
Myeloid Leukemia(1), and Philadelphia negative myeloproliferative
neoplasm ;polycythemia vera, Essential thrombocythemia, myelofibrosis,
profibrotic myelofibrosis (2,3).
Essential thrombocythemia is the most common type of myeloproliferative
neoplasms. The cause of ET is the overproduction of hematopoietic cells
due to mutation of JAK2, CALR or MPL genes. 55% of essential
thrombocythemia have JAK2 mutation (2), essential thrombocythemia is
usually scattered but it was found to be familial in rare cases in
different parts of the world (4).
Coronary artery disease including acute coronary syndrome; it refers to
a variety of clinical presentations ranging from those for ST-segment
elevation myocardial infarction to presentation found in non-ST-segment
elevation myocardial infarction or in unstable angina (5).
Case presentation
A 30-year-old Egyptian male; nonsmoker, with no previous medical
illnesses, presented to the Emergency Room complaining of chest pain.
This pain had been stabbing in nature involving the central area of the
chest, aggravated by exertion and relieved by rest, with no radiation
elsewhere.
After examination, he was not in distress, he was vitally stable, chest
was clear, normal heart sounds. Laboratory findings state that Hemoglobin
levels are within normal limits along with is White cell counts while
his platelets are slightly above the upper limit (table1).
Cardiac enzymes were elevated, electrocardiography showed significant ST
segment changes, echocardiography showed normal ejection fraction with
no valve lesions and no wall motion abnormality.
Patient was diagnosed with Non ST-segment elevation myocardial
infarction and sent to
Cath lab where he was found to have 90% stenosis in the proximal part
of left anterior descending artery, Stent was placed and patient was
discharged on anti-ischemic medications
Four years after cardiac event, patient referred to hematology clinic
because of persistent thrombocytosis. He was seen in hematology clinic;
work up for essential thrombocytosis was done. JAK2 mutation was
positive and consistent with myeloproliferative neoplasms
Moreover, bone marrow biopsy was done and was consistent with essential
thrombocytosis. He received aspirin which made his platelet counts
improve.
(Figure 1) platelets before initiation of treatment
(Figure 2) platelets after initiation of treatment
Discussion
According to the world health organization diagnosis of essential
thrombocythemia requires 4 major criteria (a-Platelet count ≥ 450 ×
109/L, B- Bone marrow picture consistent with essential thrombocythemia,
C- Not meeting world health organization criteria for BCR- ABL1 +
chronic myeloid leukemia, Polycythemia vera, primary myelofibrosis,
myelodysplastic syndrome or other myeloid neoplasm, D- Presence of JAK2,
CALR or MPL mutation, or presence of 3 major criteria and 1 minor
criteria (absence of evidence for reactive thrombocytosis)” (6).
We consider four risk categories in essential thrombocythemmia; very low
(age ≤ 60 years, no thrombosis history, JAK2 wild-type), low (same as
very low but JAK2 mutation present), intermediate (age > 60
years, no thrombosis history, JAK2 wild-type) and high (thrombosis
history present or age > 60 years with JAK2 mutation) (6).
The patient with absent risk factors of thrombosis, and borderline
platelet count, push us to think of essential thrombocythemia as the
cause of thrombotic events when there is absence of other risk factors
regardless of how much platelet count is elevated.
Commonly speaking, coronary syndrome is one of the thrombotic diseases,
and it is crucial to identify the risk factors for it. Essential
thrombocythemia is one of the risk factors for developing ACS, which
should not be missed in patients presenting with acute coronary syndrome
especially those with absent other risk factors.
Most of the patients who present with acute coronary syndrome with
background of essential thrombocythemia have few or no other risk
factors for acute coronary syndrome, in comparison to other acute
coronary syndrome causes, and this is because the events are mainly due
to thrombosis.
The risk of developing acute coronary syndrome in patient with essential
thrombocythemia is not uncommon (7). As most of cardiologist with focus
on treating of the cardiac even and with not give attention to the risk
factors which resulting in missed diagnosis of essential thrombocythemia
especially in patient with borderline platelet count.
Many patients with borderline platelets count pass unnoticed, they would
come for many other reasons to the health care facility and leave
without further work up. The patient we include in this study came in
many occasions with borderline platelets count and one of these was with
major complication of the disease, however he was missed by the
physicians. The reason for that could be the shortage of awareness about
the new criteria for diagnosis, which leads to under estimation, as many
physicians will not put essential thrombocythemia in their mind until
they see a big rise in platelet count.
Conclusion
Taking everything into consideration, Thrombosis is one of the major
complications of essential thrombocythemia, it occurs around 50% of
patient with essential thrombocythemia at the time of diagnosis.
Initiating Treatment early will decrease the number of essential
thrombocythemia induced thrombosis. Therefore, it is important to
increase the awareness among physicians about the new world health
organization criteria for the diagnosis and
emphasizing on putting essential thrombocythemia on the top of
differential diagnosis especially in patients with no other risk factors
of thrombosis, to help catch the disease early and reduce the risk of
thrombosis.
Acknowledgement
The author would like to thank Qatar National Library for funding this
publication. We would also like to thank Internal Medicine Residency
Program at Hamad Medical Corporation for supporting research.
Ethics approval and consent to participate
Case approved by HMC Medical Research center. the patient gave his
written informed consent for his case to be Published.
Consent for publication
Written informed consent was obtained from the patient for publication
of this case report and any accompanying images. A copy of the written
consent is available for review by the Editor-in- chief of this journal
Availability of supporting data
Not applicable
Competing of interest
The authors declare that they have no competing interests
Funding Sources
Qatar National Library: Phone number: +97444540100; Fax number:
+974445440401; Email address: qnl@qnl.qa
Authors’ Contribution
Mohammad K Alzyod: writing and editing
Ahmad matarneh: Review and corresponding author
Mohamed A. Yassin: writing and editing
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