Myeloproliferative neoplasms (MPN) are clonal diseases of hematopoietic stem cells
characterized by the hyperplasia of one or more myeloid cell lines that retain their
capacity for terminal differentiation [
[1]
]. Three entities that share overlapping but distinct clinical and laboratory characteristics
are referred to as Philadelphia chromosome-negative (or BCR-ABL-negative) MPN: polycythemia
vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The incidence
of ET in Europe is estimated to be 0.4 to 1.7 per 100,000 person-years [
[2]
]. As all BCR-ABL-negative MPN, ET carries an increased risk of thrombosis and bleeding.
A thromboembolic event can complicate the course of ET but more frequently can be
its mode of presentation [
[3]
]. The risk of arterial and venous thrombotic events in patients with MPN is estimated
to be respectively 4.9 and 6.7 times higher than in the general population [
[4]
]. The overall risk of arterial or venous thrombosis in ET varies between 12% and 21%
[
5
,
6
,
7
]. Of note, arterial and venous thrombosis are observed in 14% and 8% of patients before
or at ET diagnosis and may occur in another 15% and 8% of patients during a median
follow-up of 9.9 years [
[8]
]. Our clinical experience as well as published data indicate that thromboembolic events
are frequent at the moment of diagnosis, but may also occur long before the presence
of ET is recognized [
[9]
].Keywords
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References
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- Bleeding, thrombosis, and anticoagulation in myeloproliferative neoplasms (MPN): analysis from the German SAL-MPN-registry.J Hematol Oncol. 2016; 9: 18
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Article info
Publication history
Published online: September 15, 2022
Accepted:
September 6,
2022
Received:
August 28,
2022
Identification
Copyright
© 2022 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
