Essential thrombocythemia: a hemostatic view of thrombogenic risk factors and prognosis

Maleknia, Mohsen; Shahrabi, Saeid; Ghanavat, Majid; Vosoughi, Tina; Saki, Najmaldin

doi:10.1007/s11033-020-05536-x

Cited by 8 publications

(11 citation statements)

References 95 publications

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“…The main clinical manifestations are increased incidence of thromboembolic and bleeding events[ 1 ]. Research shows that thrombotic complications are the main factors affecting mortality in essential thrombocythemia (ET) patients[ 2 ], and the incidence of major haemorrhagic complications was very low in comparison with that of thrombotic episodes. These complications are most common in cases of ischaemia caused by arterial thrombosis, followed by venous thrombosis and microcirculation disorders[ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Essential thrombocythemia with non-ST-segment elevation myocardial infarction as the first manifestation: A case report

Wang¹,

Chen²,

Wu³

et al. 2022

WJCC

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BACKGROUND We report a case of essential thrombocythemia (ET) in a 44-year-old male who exhibited non-ST-segment-elevation myocardial infarction (NSTEMI) as the first manifestation without known cardiovascular risk factors (CVRFs). For the first time, we reported a left main trifurcation lesion in NSTEMI caused by ET, including continuous stenosis lesions from the left main to the ostial left anterior descending (LAD) artery and an obvious thrombotic lesion in the ostial and proximal left circumflex (LCX) artery. There was 60% diffuse stenosis in the left main (LM) that extended to the ostial LAD, thrombosis of the ostial LAD and proximal LCX, and 90% stenosis in the proximal LCX. During the operation, thrombus aspiration was performed, but no obvious thrombus was aspirated. Performing the kissing balloon technique (KBT) in the LCX and LM unexpectedly increased the narrowness of the LAD. Then, the single-stent crossover technique, final kissing balloon technique and proximal optimization technique (POT) were performed. On the second day after percutaneous coronary intervention (PCI), the number of platelets (PLTs) still increased significantly to as high as 696 × 10 9 /L. The bone marrow biopsy done later, together with JAK2 (exon 14) V617F mutation, confirms the diagnosis of ET. Hydroxyurea was administered to inhibit bone marrow proliferation to control the number of PLTs. CASE SUMMARY A 44-year-old male patient went to a local hospital for treatment for intermittent chest pain occurring over 8 h. The examination at the local hospital revealed elevated cTnI and significantly elevated platelet. Then, he was diagnosed with acute myocardial infarction and transferred to our hospital for emergency interventional treatment by ambulance. During the operation, thrombus aspiration, the single-stent crossover technique, final kissing balloon technique and POT were performed. Dual antiplatelet therapy comprising aspirin and ticagrelor was used after PCI. Evidence of mutated JAK2 V617F and bone marrow biopsy shown the onset of ET. Together with JAK2 (exon 14) V617F mutation, ET was diagnosed according to the World Health Organization (WHO) diagnostic criteria, and the patient was placed on hydroxyurea. During the one-year postoperative period, repeated examinations showed a slight increase in PLTs, but the patient no longer had chest tightness, chest pain or bleeding or developed new thromboembolisms. CONCLUSION Routine physical examinations and screenings are conducive to the early detection of ET, and the risk for thrombosis should be assessed. Then, active antiplatelet therapy and myelosuppression therapy should be used for high-risk ET patients.

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Section: Introductionmentioning

confidence: 99%

Essential thrombocythemia with non-ST-segment elevation myocardial infarction as the first manifestation: A case report

Wang¹,

Chen²,

Wu³

et al. 2022

WJCC

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“…Essential thrombocythemia (ET) is a type of acquired stem cell-derived clonal disease associated with the expansion of megakaryocytic (MK)/platelet line. ET has been categorized as a myeloproliferative neoplasm (MPN) by Dameshech since 1954 [1,2]. This neoplasm's primary cause is the hyperproliferation of hematopoietic cells (HSCs) due to genetic driver mutations po-tentiating the myeloproliferative processes.…”

Section: Introductionmentioning

confidence: 99%

“…It is noteworthy that studies have shown that approximately 10% to 15% of ET patients do not carry common driver mutations (Triple-Negative patients). The results of these studies indicate that misregulation in the JAK-STAT pathway and upregulation of genes involved in platelet proliferation, including ITGA2B and ITGB3a, lead to the formation of differential gene expression profiles in Triple-Negative patients www.opastonline.com [2]. Studies show that 100-205 individuals per 100,000 suffering from MPN or ET annually [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…The results of these studies indicate that misregulation in the JAK-STAT pathway and upregulation of genes involved in platelet proliferation, including ITGA2B and ITGB3a, lead to the formation of differential gene expression profiles in Triple-Negative patients www.opastonline.com [2]. Studies show that 100-205 individuals per 100,000 suffering from MPN or ET annually [2][3][4]. Thrombotic events or thrombosis in the cerebral, hepatic, or coronary vessels due to the platelet defect is the leading cause of death in ET patients [5].…”

Section: Introductionmentioning

confidence: 99%

“…A few patients may have other mutations, including MPL exon 10 mutation, reported in approximately 3-15% of ET and PMF patients [9]. Since about 20% of ET patients are negative for these three mutations (Triple-Negative patients), the absence of JAK2, CALR, and MPL mutations does not rule out the ET presence [2,10].…”

Section: Introductionmentioning

confidence: 99%

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PEAR1 Genetic Variants in Essential Thrombocythemia: The Prevalence and Association with Hematological Parameters and ET Mutations

2022

JGEBR

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Objective: Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm characterized by the expansion of the megakaryocytic/platelet line. Given the undeniable role of genetic variations in the pathogenesis of ET, as well as the proven effects of PEAR1 SNPs on platelet function, the innovative purpose of this study is to investigate the prevalence of PEAR1 variants (rs12041331 and rs12566888)and their relationship to hematological parameters and ET-related mutations. Materials and Methods: We studied 105 ET patients and analyzed ET patients’ mutational profiles, including JAK2 V617F mutation (detected by Allele-specific PCR), CALR, and MPL mutations (both through PCR amplification). Two SNPs of the PEAR1 gene were assessed through ARMS-PCR, and the Sanger method was used for the validation of ARMS-PCR amplification. Results: The prevalence of rs12041331 and rs12566888 in ET patients were 43.9% and 38.5%, respectively, and rs12041331 was significantly associated with increased platelet counts (P-Value: 0.02). As expected, the incidence of thrombotic events in JAK2+ patients was high and significantly associated with JAK2 mutation (P-Value: 0.02). The prevalence of thrombotic events was also high in patients with the rs12041331 variant. Besides, a significant relationship was also found between the rs12041331 and CALR mutation (P-Value: 0.03). Conclusions: In recent years, the footprint of the PEAR1 variant’s effect on platelet aggregation led to evaluating these variants in ET patients. Finally, the significant relationship between the rs12041331 variant and increased platelet count and CALR mutation announced that the idea of this study could be pursued and challenged in the future.

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The role of leukocytes in myeloproliferative neoplasm thromboinflammation

Liu,

Wang,

Huang

et al. 2024

Journal of Leukocyte Biology

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Classic myeloproliferative neoplasms lacking the Philadelphia chromosome are stem cell disorders characterized by the proliferation of myeloid cells in the bone marrow and increased counts of peripheral blood cells. The occurrence of thrombotic events is a common complication in myeloproliferative neoplasms. The heightened levels of cytokines play a substantial role in the morbidity and mortality of these patients, establishing a persistent proinflammatory condition that culminates in thrombosis. The etiology of thrombosis remains intricate and multifaceted, involving blood cells and endothelial dysfunction, the inflammatory state, and the coagulation cascade, leading to hypercoagulability. Leukocytes play a pivotal role in the thromboinflammatory process of myeloproliferative neoplasms by releasing various proinflammatory and prothrombotic factors as well as interacting with other cells, which contributes to the amplification of the clotting cascade and subsequent thrombosis. The correlation between increased leukocyte counts and thrombotic risk has been established. However, there is a need for an accurate biomarker to assess leukocyte activation. Lastly, tailored treatments to address the thrombotic risk in myeloproliferative neoplasms are needed. Therefore, this review aims to summarize the potential mechanisms of leukocyte involvement in myeloproliferative neoplasm thromboinflammation, propose potential biomarkers for leukocyte activation, and discuss promising treatment options for controlling myeloproliferative neoplasm thromboinflammation.

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Essential thrombocythemia: a hemostatic view of thrombogenic risk factors and prognosis

Cited by 8 publications

References 95 publications

Essential thrombocythemia with non-ST-segment elevation myocardial infarction as the first manifestation: A case report

Essential thrombocythemia with non-ST-segment elevation myocardial infarction as the first manifestation: A case report

PEAR1 Genetic Variants in Essential Thrombocythemia: The Prevalence and Association with Hematological Parameters and ET Mutations

The role of leukocytes in myeloproliferative neoplasm thromboinflammation

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