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

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Abstract

Essential thrombocythemia (ET) is a classical myeloproliferative neoplasm that is susceptible to hypercoagulable state due to impaired hemostatic system, so that thrombotic complications are the leading cause of mortality in ET patients. The content used in this article has been obtained by the PubMed database and Google Scholar search engine from English-language articles (2000–2019) using the following keywords: "Essential thrombocythemia," "Thrombosis," "Risk factors" and "Hemostasis. In this neoplasm, the count and activity of cells such as platelets, leukocytes, endothelial cells, as well as erythrocytes are increased, which can increase the risk of thrombosis through rising intercellular interactions, expression of surface markers, and stimulation of platelet aggregation. In addition to these factors, genetic polymorphisms in hematopoietic stem cells (HSCs), including mutations in JAK2, CALR, MPL, or genetic abnormalities in other genes associated with the hemostatic system may be associated with increased risk of thrombotic events. Moreover, disruption of coagulant factors can pave the way for thrombogeneration. Therefore, the identification of markers related to cell activation, genetic abnormalities, or alternation in the coagulant system can be used together as diagnostic and prognostic markers for the occurrence of thrombosis among ET patients. Thus, because thrombotic complications are the main factors of mortality in ET patients, a hemostatic viewpoint and risk assessment of cellular, genetic, and coagulation factors can have prognostic value and contribute to the choice of effective treatment and prevention of thrombosis.

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Abbreviations

WBCs:

White blood cells

RBCs:

Red blood cells

ROS:

Reactive oxygen species

MPV:

Mean platelet volume

TXA2:

Thromboxane-A2

ADP:

Adenosine diphosphate

sCD40L:

Soluble- CD40 ligand

MPs:

Microparticles

vWF:

Von Willebrand factor

ECs:

Endothelial cells

TM:

Thrombomodulin

PAI-1:

Plasminogen- activator inhibitor

TG:

Thrombin generation

4G:

4-Guanosine sequence

5G:

5-Guanosine sequence

TF:

Tissue factor

NOS3:

Nitric oxide synthase-3

NO:

Nitric oxide

MTHF:

Methylenetetrahydrofolate reductase

TFPI:

Tissue factor pathway inhibitor

CAL:

Calreticulin

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Acknowledgements

We wish to thank all our colleagues in Allied Health Sciences School, Ahvaz Jundishapur University of Medical Sciences.

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Authors and Affiliations

  1. Thalassemia & Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

    Mohsen Maleknia, Tina Vosoughi & Najmaldin Saki

  2. Student Research Committee, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

    Mohsen Maleknia

  3. Department of Clinical Laboratory Sciences, School of Allied Medical Sciences, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

    Mohsen Maleknia & Najmaldin Saki

  4. Department of Biochemistry and Hematology, Faculty of Medicine, Semnan University of Medical Sciences, Semnan, Iran

    Saeid Shahrabi

  5. Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran

    Majid Ghanavat

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  1. Mohsen Maleknia
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  2. Saeid Shahrabi
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  3. Majid Ghanavat
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  4. Tina Vosoughi
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  5. Najmaldin Saki
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Dr. NS conceived the manuscript and revised it. MM, SS, MG, and TV wrote the manuscript and prepared tables and figures.

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Correspondence to Najmaldin Saki.

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Maleknia, M., Shahrabi, S., Ghanavat, M. et al. Essential thrombocythemia: a hemostatic view of thrombogenic risk factors and prognosis. Mol Biol Rep 47, 4767–4778 (2020). https://doi.org/10.1007/s11033-020-05536-x

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  • DOI: https://doi.org/10.1007/s11033-020-05536-x

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