The age distribution of individuals impacted by myeloproliferative neoplasms (MPNs) is notably diverse, as articulated by Ruben Mesa, MD, FACP, the executive director of Atrium Health Wake Forest Baptist Comprehensive Cancer Center and president of Atrium Health Levine Cancer.
With younger patients, it becomes paramount to comprehend the heightened risk associated with disease progression, particularly in light of their anticipated long-term coexistence with the illness. This situation is further complicated by the implications that various therapies may have on their fertility.
This transcript was lightly edited for clarity.
Transcript
About 20% of patients with myeloproliferative neoplasms are in the adolescent to young adult population. Are there characteristics that differentiate this younger population from older ones or treatment considerations that differ among the age groups?
I would assert that while the median age for diagnosis tends to be in the 60s, the distribution of affected individuals is quite expansive. Unlike other conditions that may show a pronounced concentration in older age groups, MPNs demonstrate a surprisingly broad spectrum of age distribution. Notably, early phases of MPN such as essential thrombocythemia (ET) and polycythemia vera (PV) frequently present in individuals in their 30s, 40s, and 50s. Though the incidence among teenagers and those in their 20s—the AYA [adolescent and young adult] demographic—is relatively rare, recent observations indicate a greater frequency than previously recognized, underscoring the necessity of awareness regarding this broader demographic impact.
Clearly, with younger individuals, we’re keenly aware of several vital considerations. Firstly, the duration of their disease raises concerns regarding the risk of progression to a more advanced myeloid neoplasm; this risk significantly escalates the longer they remain afflicted. Specifically, individuals who have lived with the disease for a decade or longer face increasing risks of conditions such as myelofibrosis, which can be life-threatening. In contrast, ET and PV are typically more manageable, allowing for maintenance of survival. Thus, disease progression poses serious concerns, especially for younger patients who will experience prolonged exposure.
Additionally, there are significant implications regarding both fertility preservation and the careful selection of medical therapies. Historically, hydroxyurea has been widely employed in the treatment of ET and PV, yet this medication is contraindicated during pregnancy, which necessitates a careful consideration of treatment options. As a result, alternatives such as interferons or long-acting interferons are often favored for this younger patient population. Not only do these alternatives have fewer fertility implications, but there is also emerging evidence suggesting that interferons may effectively slow disease progression, making them particularly relevant for younger individuals facing these health challenges.
**Interview with Dr. Ruben Mesa on Myeloproliferative Neoplasms**
**Interviewer:** Dr. Mesa, thank you for joining us today to discuss myeloproliferative neoplasms, or MPNs. It’s evident that a significant portion of patients affected by MPNs is relatively young. Can you elaborate on the age distribution of these patients?
**Dr. Mesa:** Absolutely, and thank you for having me. While the median age for diagnosis of MPNs generally falls in the 60s, we see a noteworthy 20% of patients who are adolescents or young adults. This diverse age distribution is somewhat unique when compared to other malignancies, which tend to be more concentrated in older populations.
**Interviewer:** That’s interesting. With younger patients, what are some unique challenges they face with MPNs, especially regarding treatment?
**Dr. Mesa:** Young patients face distinct challenges, especially concerning the long-term implications of disease progression. Since they are likely to live with MPNs for a prolonged period, it’s important to understand the potential risks tied to their specific condition, such as fertility issues related to therapy. The medical management of MPNs in younger patients necessitates a nuanced approach that balances effective treatment with quality of life considerations.
**Interviewer:** Given the diversity in age and the potential complications regarding treatment, are there specific treatment considerations that differentiate younger patients from older ones?
**Dr. Mesa:** Yes, indeed. While older patients often have different comorbidities that can affect their treatment options, younger patients may require a more tailored therapeutic strategy. Not only must we consider the efficacy of treatments, but we also need to evaluate their long-term impact on health and lifestyle, particularly regarding fertility and family planning.
**Interviewer:** That’s critical information. As a community, how can we better support young patients diagnosed with MPNs?
**Dr. Mesa:** Awareness is key. We need to foster a deeper understanding among healthcare providers about the unique challenges faced by younger patients. Additionally, creating supportive networks and resources that address both the medical and psychosocial aspects of living with MPNs can empower these patients to manage their disease more effectively.
**Interviewer:** Thank you, Dr. Mesa, for shedding light on these important issues regarding myeloproliferative neoplasms, particularly for the younger population. Your insights are invaluable.
**Dr. Mesa:** Thank you for the opportunity to discuss this vital topic.
