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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