Thrombosis, Major Bleeding Events More Frequent in Myelofibrosis Compared With Other Myeloproliferative Neoplasms

March 26, 2025

Author(s): Luke Halpern, Assistant Editor

Results of a nationwide Swedish population-based study indicate that patients with myelofibrosis (MF) have increased rates of thromboembolic events and major bleeding compared with presentations in other myeloproliferative neoplasms (MPN), with thromboembolic complications and major bleeding events diverging based on varying treatment groups. These results, published by study authors in Blood Advances, can help health care providers more effectively care for patients with MF, allowing them to counsel patients on associated bleeding risks.1

Thrombosis can be a deadly complication in patients with myelofibrosis. | Image Credit: © Matthieu – stock.adobe.com

The clinical presentation of MF evolves throughout the development of the disease, eventually presenting as bone marrow fibrosis, hepatosplenomegaly, fatigue, and progressive pancytopenia, ultimately leading to reduced patient quality of life and heightened morbidity. Bleeding and thromboembolic events have been known to be possible complications of MF, but literature on this association is lacking. According to the present investigators, studies analyzing this association are plagued by small cohorts, seldom-included control patients, and populations containing varying MPNs, rather than MF specifically.2,3

To fill the present gaps in literature regarding thromboembolic and bleeding event prevalence in patients with MF, the current study authors conducted a nationwide analysis to assess the frequency of arterial and venous events, major bleeding, all-cause stroke, and all-cause mortality in Swedish patients with primary MF (PMF) and secondary MF (SMF) compared with matched controls. They also aimed to investigate if outcomes varied based on the therapy used in MF while attempting to describe relevant risk factors for major bleeding and thromboembolic events among patients with MF.1

Multiple Swedish registries of patients diagnosed with hematologic malignancies were consulted, with all adult patients registered with a diagnosis of MF from 2008 to 2021 included. In total, 1079 patients with MF and 395 controls were included, with a median age of 72 years at diagnosis. Notably, over a third of the patients (40.7%) had an International Prognostic Scoring System (IPSS) score of intermediate-2 or high risk. Furthermore, mutations were present in many patients enrolled in the study and diagnosed after June 1, 2016, with 53.6% having a JAK2 V617F mutation.1

Across the follow-up period, 125 arterial and 51 venous events occurred in the MF cohort, with event rates of 2.59 and 1.06 events per 100 patient years, compared with 337 (rate 1.51, HR: 1.73; 95% CI, 1.40-2.12; < .001) and 86 (rate 0.38, HR: 2.75; 95% CI, 1.95-3.90; P < .001) among the control patients. When patients were divided based on diagnoses of PMF or SMF, rates of arterial and venous events were noticeably higher among patients with SMF compared with PMF. In addition, 80 cases of acute myocardial infarction (rate: 1.66), 40 ischemic strokes (rate: 0.83), and 38 pulmonary emboli (rate: 0.79) were documented in the MF cohort.1

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Management of Bleeding, Thrombotic and Pregnancy-Related Complications in Women with Myeloproliferative Neoplasms: A Case-Based Review Focusing on Sex-Specific Challenges

February 25, 2025

by Thita Chiasakul and Ross I. Baker

Abstract

Myeloproliferative neoplasms (MPNs) are a heterogeneous group of clonal hematopoietic disorders that pose unique challenges in women, particularly regarding thrombosis, bleeding, fertility, and pregnancy. Women with MPN exhibit distinct thrombotic and sometimes contradictory bleeding profiles, including a higher prevalence of unusual thrombosis such as cerebral and splanchnic vein thrombosis and increased risk of hemorrhage from anti-thrombotic medication, acquired von Willebrand syndrome and platelet dysfunction. Estrogen-containing contraceptives should generally be avoided due to thrombotic risk. Around 10–20% of newly diagnosed MPN cases are women of childbearing age and the number is increasing annually. MPN patients when compared to controls have a lower rate of live birth rate of 71% vs. 80% with a hazard ratio of 0.78 (95% CI: 0.68–0.90), and increased preterm birth (14% vs. 4%), low birth weight (<2500 g, 10% vs. 4%), and increased cesarean section rate (32% vs. 17%). Management of MPN-related pregnancy requires specific considerations regarding the prevention of thrombosis, bleeding, and pregnancy-related complications. Management strategies during pregnancy include low-dose aspirin and consideration of low-molecular-weight heparin and interferon. Despite these challenges, most women with MPN can achieve successful pregnancies with optimized care. In this case-based review, we present two cases that illustrate key aspects of managing MPN in women, summarize the current literature, and propose a diagnostic and management framework tailored to these complexities.

Assessing and Managing Bleeding Risk Pre-Surgery in Patients With MPNs

August 20 , 2024

Risk factors for bleeding and thrombosis among patients with myeloproliferative neoplasms (MPNs) who are scheduled for surgery are multifaceted and require consideration of individual patient characteristics, risk assessment, and perioperative management, according to results published in Cureus.

“Thrombosis, venous or arterial, is a major cause of mortality and morbidity in [essential thrombocythemia (ET)] and [polycythemia vera], while bleeding is more concerning in [myelofibrosis] and ET,” Mihaela Andreescu, MD, PhD, and colleagues wrote. “Surgical procedures also pose a significant risk for bleeding in MPNs, with a probability of 7.2% during surgery. Assessing bleeding and thrombosis risk in patients scheduled for surgery is crucial to optimize patient outcomes.”

Specific Risks for Bleeding and Thrombosis

Risk assessment tools included rational elastrometry (ROTEM), International Predictive Score for Thrombosis in ET (IPSET), and the dynamic international prognostic scoring system (DIPSS).

The researchers identified age (>60), history of thrombosis, and genetic mutations, particularly variants of JAK2V617F, as risk factors for thrombosis in patients with MPN. Risk factors for bleeding included leukocytosis, thrombocytosis, acquired von Willebrand syndrome, and history of bleeding.

“Individual patient factors must be considered to minimize severe bleeding and thrombotic complications in surgeries,” Dr. Andreescu and colleagues wrote. “Risk assessment and perioperative management are important aspects of improving the QOL and preventing complications in surgeries.”

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A Review About the Assessment of the Bleeding and Thrombosis Risk for Patients With Myeloproliferative Neoplasms Scheduled for Surgery

Mihaela Andreescu
Bogdan Andreescu

Published March 12, 2024

Abstract

Myeloproliferative neoplasms (MPNs) present a unique challenge in surgical management due to their inherent predisposition to both bleeding and thrombosis. MPNs are a heterogenous group of acquired clonal conditions. The three classic MPNs are essential thrombocythemia (ET), myelofibrosis (PMF), and polycythemia vera (PV). All subtypes of MPN are associated with both thrombotic and bleeding complications. There are four risk categories for thrombosis in MPN patients: age, thrombosis history, and JAK-2 mutation. They are further classified as very low, low, intermediate, and high risk. The genetic landscape of MPN is fascinating and complex like all myeloid disorders. Bleeding risk can be assessed through leukocytosis, thrombocytosis, acquired von Willebrand syndrome (AVWS), and a previous history of bleeding in a patient. Risk assessment and perioperative management are important aspects of improving the quality of life and preventing complications in surgeries. Preoperative management includes a risk assessment of venous thromboembolism, use of appropriate pharmacological treatment, platelet count control, and correction and cardiovascular risk factors. This review summarizes the assessment of bleeding and thrombosis risk for patients with MPNs scheduled for surgery. Furthermore, this review discusses various tools that can be used to identify MPN patients at risk of thrombosis prior to surgery.

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