Advances in the Prevention of Thrombosis in Myeloproliferative Neoplasms

A special issue of Hemato (ISSN 2673-6357). This special issue belongs to the section "Chronic Myeloid Disease".

Deadline for manuscript submissions: closed (31 March 2021) | Viewed by 9911

Special Issue Editors


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Guest Editor
Hematology Institute, Meir Medical Center, 59 Tchernichovsky St, Kfar Saba 44281, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
Interests: thromboembolism in myeloproliferative neoplasms; optimizing direct oral anticoagulant use across patient subgroups

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Guest Editor
Department of Hematology, Guy’s and St. Thomas’ NHS Foundation Trust, London, UK
Interests: myeloproliferative neoplasms; essential thrombocythaemia; polycythaemia vera; myelofibrosis; eosinophilia and nuetrophilia; MPN/MDS overlap; clinical trials

Special Issue Information

Dear Colleagues,

This Hemato Special Edition, entitled “Advances in the prevention of thrombosis in myeloproliferative neoplasms”, is dedicated to providing up-to-date information regarding both primary and secondary thrombosis prophylaxis in patients with polycythemia vera, essential thrombocythemia and primary myelofibrosis.

Thromboembolism is the most important modifiable complication in patients with myeloproliferative neoplasms (MPNs) and thrombotic episodes occur throughout the disease course despite current prophylactic strategies. Importantly, thrombosis is the leading cause of death in MPN, thus improvement in our ability to prevent these events is warranted. Areas that will be highlighted in the Special Issue will include advances in thrombosis risk stratification in MPNs, novel approaches to primary prophylaxis, including the addition of the newly available direct oral anticoagulants to available drugs, the optimization of aspirin dosing using a twice-daily versus once-daily regimen and lessons learned regarding prolonged secondary prophylaxis particularly for intra-abdominal thrombosis. Data will also be presented regarding the level of thrombosis risk and appropriate management strategies for each of the individual MPNs.

Prof. Dr. Martin Ellis
Prof. Dr. Claire Harrison
Guest Editors

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Keywords

  • myeloproliferative neoplasms
  • thrombosis risk
  • prophylaxis
  • anticoagulation

Published Papers (4 papers)

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Review

12 pages, 247 KiB  
Review
Is There a Role for Direct Oral Anticoagulants in the Primary and Secondary Prevention of Myeloproliferative Neoplasm Associated Thrombosis?
by Uzma Faruqi and Karen A. Breen
Hemato 2021, 2(4), 769-780; https://doi.org/10.3390/hemato2040053 - 14 Dec 2021
Viewed by 2362
Abstract
Philadelphia chromosome negative myeloproliferative neoplasms (MPN) are clonal haematopoietic stem cell disorders. Of the MPNs, polycythaemia vera (PV) and essential thrombocythaemia (ET) confer a high thrombotic risk which may be the presenting feature of the disease. Thrombotic complications consist of both arterial and [...] Read more.
Philadelphia chromosome negative myeloproliferative neoplasms (MPN) are clonal haematopoietic stem cell disorders. Of the MPNs, polycythaemia vera (PV) and essential thrombocythaemia (ET) confer a high thrombotic risk which may be the presenting feature of the disease. Thrombotic complications consist of both arterial and venous events and the presence of the JAK2 V617F mutation is associated with higher risk. Patients presenting with an unprovoked thrombus, particularly at an unusual site, e.g., splanchnic circulation, should be screened for the presence of this mutation. Historically, warfarin has been the only option for oral anticoagulation; however, there is now increasing evidence and practise to use direct oral anticoagulants (DOACs) in cancer. The seminal randomised control trials have demonstrated non-inferiority compared to low molecular weight heparin (LMWH) with a preferable bleeding profile. DOACs are now the first line treatment for atrial fibrillation and venous thromboembolic disease, as recommended by NICE, and therefore there is increasing familiarity with these agents. Furthermore, there are now targeted antidotes available. This paper reviews evidence for efficacy and safety of DOACs in MPN. Whilst no randomised control trials have been performed, several retrospective studies and reviews of registry data have reproducibly demonstrated that, alongside cytoreduction, DOACs represent an effective modality of anticoagulation for treatment of venous thromboembolism in MPN. Furthermore, dosing regimens provide the option for longer term secondary prophylaxis. Use of DOACs in arterial thrombosis is an area for future development and there is already some evidence for utility in peripheral vascular disease. Full article
11 pages, 260 KiB  
Review
The Approach to Thrombosis Prevention across the Spectrum of Philadelphia-Negative Classic Myeloproliferative Neoplasms
by Steffen Koschmieder
Hemato 2021, 2(3), 392-402; https://doi.org/10.3390/hemato2030025 - 30 Jun 2021
Cited by 2 | Viewed by 2422
Abstract
Patients with myeloproliferative neoplasm (MPN) are potentially facing diminished life expectancy and decreased quality of life, due to thromboembolic and hemorrhagic complications, progression to myelofibrosis or acute leukemia with ensuing signs of hematopoietic insufficiency, and disturbing symptoms such as pruritus, night sweats, and [...] Read more.
Patients with myeloproliferative neoplasm (MPN) are potentially facing diminished life expectancy and decreased quality of life, due to thromboembolic and hemorrhagic complications, progression to myelofibrosis or acute leukemia with ensuing signs of hematopoietic insufficiency, and disturbing symptoms such as pruritus, night sweats, and bone pain. In patients with essential thrombocythemia (ET) or polycythemia vera (PV), current guidelines recommend both primary and secondary measures to prevent thrombosis. These include acetylsalicylic acid (ASA) for patients with intermediate- or high-risk ET and all patients with PV, unless they have contraindications for ASA use, and phlebotomy for all PV patients. A target hematocrit level below 45% is demonstrated to be associated with decreased cardiovascular events in PV. In addition, cytoreductive therapy is shown to reduce the rate of thrombotic complications in high-risk ET and high-risk PV patients. In patients with prefibrotic primary myelofibrosis (pre-PMF), similar measures are recommended as in those with ET. Patients with overt PMF may be at increased risk of bleeding and thus require a more individualized approach to thrombosis prevention. This review summarizes the thrombotic risk factors and primary and secondary preventive measures against thrombosis in MPN. Full article
24 pages, 918 KiB  
Review
Can Novel Insights into the Pathogenesis of Myeloproliferative Neoplasm-Related Thrombosis Inform Novel Treatment Approaches?
by Ofir Wolach and Adi Shacham Abulafia
Hemato 2021, 2(2), 305-328; https://doi.org/10.3390/hemato2020018 - 16 May 2021
Cited by 3 | Viewed by 2665
Abstract
Despite recent advances in diagnosis and therapy, arterial and venous thrombosis remain a major cause of morbidity and mortality in Philadelphia-negative myeloproliferative neoplasms (MPNs). Preventing and treating arterial and venous thrombosis represent one of the major goals in MPNs. The prothrombotic phenotype of [...] Read more.
Despite recent advances in diagnosis and therapy, arterial and venous thrombosis remain a major cause of morbidity and mortality in Philadelphia-negative myeloproliferative neoplasms (MPNs). Preventing and treating arterial and venous thrombosis represent one of the major goals in MPNs. The prothrombotic phenotype of MPNs is the result of a complex interplay between several components. Neutrophils, platelets, red blood cells (RBCs) and endothelial cells assume an activated phenotype in MPNs and undergo morphologic and metabolic changes that render these cells prothrombotic. These changes are in part the result of alterations induced by MPN initiating, driving mutations as well as the effect of extrinsic factors that stem from cell interactions as well as the inflammatory environment and rheological properties that characterize MPNs. In this review, we address current management issues in MPNs and provide an update on recent understanding of the pathogenesis of thrombosis in MPNs. We also address how lessons learned from other thrombo-inflammatory conditions can further inform and improve management of thrombosis in MPNs. Based on the above data and recent discoveries and developments, we discuss potential novel targets and therapeutic approaches to tackle the challenge of thrombosis in MPNs. Full article
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9 pages, 334 KiB  
Review
Anticoagulation for Splanchnic Vein Thrombosis in Myeloproliferative Neoplasms: The Drug and the Duration
by Wafik G. Sedhom and Brady Lee Stein
Hemato 2021, 2(2), 255-263; https://doi.org/10.3390/hemato2020015 - 12 May 2021
Cited by 1 | Viewed by 1797
Abstract
Myeloproliferative neoplasms are a common cause of splanchnic vein thrombosis, which causes significant morbidity and mortality. Indefinite anticoagulation is the mainstay of therapy, and vitamin K antagonists (VKAs) are routinely used since hematologists have the most experience with this drug class. The role [...] Read more.
Myeloproliferative neoplasms are a common cause of splanchnic vein thrombosis, which causes significant morbidity and mortality. Indefinite anticoagulation is the mainstay of therapy, and vitamin K antagonists (VKAs) are routinely used since hematologists have the most experience with this drug class. The role of direct oral anticoagulants (DOACs) is promising, but still undergoing evaluation. Cytoreduction with hydroxyurea or pegylated interferon is often used when cytosis is present, but their roles are yet to be defined when the complete blood count is normal. Janus kinase (JAK) inhibition may have a complementary role in reducing splenomegaly and portal hypertension. Full article
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