Research Article
Thrombophilic Risk of Factor V Leiden, Prothrombin G20210A,
MTHFR, and Calreticulin Mutations in Essential
Thrombocythemia Egyptian Patients
Mohamed S. El-Ghonemy ,
1
Solafa El Sharawy,
1
Maryan Waheeb Fahmi,
2
Shaimaa El-Ashwah,
3
May Denewer,
3
and MA El-Baiomy
2
1
Hematology Unit, Clinical Pathology Department, Mansoura University Faculty of Medicine, Mansoura, Egypt
2
Medical Oncology Unit, Oncology Center, Mansoura University, Faculty of Medicine, Mansoura, Egypt
3
Clinical Hematology Unit, Oncology Center, Mansoura University, Faculty of Medicine, Mansoura, Egypt
Correspondence should be addressed to Mohamed S. El-Ghonemy; mohsabali@yahoo.com
Received 18 September 2019; Revised 4 December 2019; Accepted 6 February 2020; Published 30 March 2020
Academic Editor: Bashir A. Lwaleed
Copyright © 2020 Mohamed S. El-Ghonemy et al. is is an open access article distributed under the Creative Commons
Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.
Objectives. Essential thrombocythemia (ET) is one of the myeloproliferative neoplasms characterized by a sustained elevation of
platelet numbers with a tendency for thrombosis and hemorrhage. e aim of this work is to establish the relation between
calreticulin, factor V Leiden, prothrombin G20210A, and MTHFR mutations in ET patients and the thrombotic risk of these
patients. Methods. is study was carried out on 120 ETpatients and 40 apparently healthy individuals as a control group. Results.
ere were increases in WBCs, PLTcounts, PT, fibrinogen concentration factor V Leiden, and MTHFR mutation in ETpatients as
compared to the control group (P < 0.05). Also, there were increases in WBCs, PLT counts, and hematocrit value in thrombosed
ETpatients as compared to the nonthrombosed ones (P < 0.05). On the contrary, there was no significantly statistical difference in
ETpatients with JAK2 V617F positive mutation versus the JAK2 negative group (P > 0.05) and in patients with cardiovascular risk
factors versus patients with noncardiovascular risk factors (P > 0.05). ET patients with factor V Leiden, prothrombin gene, and
CALR mutations were more prone to thrombosis (odds ratio 5.6, 5.7 and 4.7, respectively). On the contrary, JAk2V 617F and
MTHFR mutations have no effect on the thrombotic state of those patients. Conclusion. ere is a significant increase risk of
thrombosis in ET patients with CALR mutation, thrombophilic mutations, as well as factor V Leiden and prothrombin gene
mutation with a risk of developing leukemic transformation.
1.Introduction
Essential thrombocythemia (ET) is one of the “myeloprolif-
erative neoplasms” (MPNs) characterized by clonal overpro-
duction of one or more blood cell lines. ETis characterized by a
sustained elevation of platelet number with a tendency for
thrombosis and hemorrhage [1]. ETmay be asymptomatic or it
may be presented by vascular occlusive events and hemor-
rhages [1, 2]. e most common molecular marker of these
diseases is JAK2 V617F mutation present in 50–70% and
calreticulin (CALR), a multifunctional calcium-binding pro-
tein, mutation present in 15–24% [1].
rombophilia is common in ET patients which may be
genetic or acquired. e pathogenesis of thrombosis in ET is
complex and not yet fully understood [2]. Several defects
have been described, including an autonomous production
of platelets unregulated by the physiologic feedback
mechanism to keep the count within the reference range [3].
Also, in ET, there were increased sensitivities to cytokines
(e.g., interleukin-3 (IL-3)) and decreased inhibition to
platelet-inhibiting factors (e.g., transforming growth factor
(TGF) beta), leading to hyperaggregation [4]. However,
Pearson [5] reported that platelet count per se does not
correlate with thrombotic incidence.
Hindawi
Advances in Hematology
Volume 2020, Article ID 7695129, 6 pages
https://doi.org/10.1155/2020/7695129