by David Wallace
A new study has shed light on the factors that may predict polycythemia vera (PV) disease progression. The REVEAL study, presented at the 2024 European Hematology Association Congress, has identified five key characteristics that could help doctors better understand which patients are at higher risk of their condition worsening over time.
Polycythemia vera is a type of blood cancer where the body produces too many red blood cells. While some patients live with stable PV for many years, others may experience progression to more severe conditions, such as myelofibrosis. Understanding who is at greater risk of progression could lead to more personalized and effective treatment strategies.
Highlights:
- REVEAL study identifies five factors associated with increased risk of polycythemia vera (PV) progression
- Factors include disease duration, history of blood clots, high white blood cell count, low hematocrit level, and genetic mutation
- 6.7% of PV patients progressed to myelofibrosis over a median follow-up of 3.7 years
- Findings may help doctors better predict disease course and tailor treatment plans
The REVEAL study, led by Dr. Michael R. Grunwald from Atrium Health’s Levine Cancer Institute, analyzed data from 2,023 PV patients treated in various U.S. medical centers between 2014 and 2019. This makes it the largest prospective observational study of PV patients to date, providing a wealth of real-world data on the disease’s behavior.
Over a median follow-up period of 3.7 years, the researchers found that 6.7% of patients progressed to myelofibrosis, a more severe bone marrow disorder. By comparing the characteristics of patients who experienced progression with those who didn’t, the team identified five factors that appeared to be associated with a higher risk of disease progression:
- Disease Duration: Patients who had been living with PV for a longer time before entering the study were more likely to experience progression.
- History of Blood Clots: Those with a previous history of thrombotic events (blood clots) showed a higher risk of disease progression.
- High White Blood Cell Count: Patients with a white blood cell count greater than 11 × 10^9/L at the start of the study were more likely to see their disease worsen.
- Low Hematocrit Level: Interestingly, patients with a hematocrit level of 0.45 L/L or lower seemed to be at higher risk. However, the researchers noted that this finding might be influenced by other factors, such as ongoing treatments.
- Genetic Mutation: A higher level of a specific genetic mutation (JAK2 p.V617F variant allele frequency) was associated with increased risk of progression.
Dr. Grunwald and his team emphasized that of these factors, the high white blood cell count and the genetic mutation level were the most consistent predictors of disease progression.
“These results provide additional support for the use of disease duration, elevated white blood cell count, and variant allele frequency as risk factors for disease progression,” Dr. Grunwald explained. “They also identify a history of blood clots as a potential new risk factor to consider.”
The study’s findings could have significant implications for PV patient care. By considering these five factors, doctors may be better equipped to predict which patients are more likely to experience disease progression. This knowledge could inform treatment decisions, potentially leading to more aggressive management for high-risk patients or more frequent monitoring to catch progression early.
It’s important to note that while these factors are associated with increased risk, they don’t guarantee that a patient’s disease will progress. Many patients with PV live for years without significant progression.
The REVEAL study also provided interesting insights into the demographics of PV patients. The median age of patients in the study was 68 years, with ages ranging from 22 to 95. Slightly more than half (51.2%) were male, and the vast majority (89.1%) were White. Most patients (82.6%) were treated in community practice settings rather than academic centers.
The study observed differences in treatment approaches between patients who experienced progression and those who didn’t. For example, patients who progressed were more likely to be receiving hydroxyurea (a medication that can help control blood cell production) at the start of the study.
While these findings represent a significant step forward in understanding PV progression, the researchers caution that more studies are needed to fully understand the complex interplay of factors influencing the disease’s course.
For patients living with polycythemia vera, this research offers hope for more personalized care in the future. As our understanding of the disease grows, so too does our ability to predict its course and tailor treatments to individual patient needs.
Reference
Grunwald M, Zwicker J, Gerds A, et al. A real-world evaluation of risk factors for disease progression in patients with polycythemia vera enrolled in REVEAL. Presented at: 2024 EHA Congress; June 13-16, 2024; Madrid, Spain. Abstract P1047.
