October 30, 2024
Author(s): Laura Joszt, MA, Mary Caffrey
The age distribution of people affected by myeloproliferative neoplasms (MPNs) is broad, explained Ruben Mesa, MD, FACP, executive director of Atrium Health Wake Forest Baptist Comprehensive Cancer Center, president of Atrium Health Levine Cancer.
With younger patients, it’s important to understand the increased risk of their disease progressing given how long they’ll live with their illness, and the impact therapies may have on 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 say that the median age is in the 60s. However, I would say that the distribution is broad. As opposed to it being a median in the 60s and there being a high concentration only in individuals that are older, it is a broader distribution. In particular the earlier phases of MPN, ET [essential thrombocythemia], and PV [polycythemia vera}, are not uncommon in those that are 30s, 40s and 50s years old. Teenagers and those in their 20s—that AYA [adolescent and young adult] population—certainly is less common, but it is more common than, I think, had been appreciated, that there’s a broader distribution affecting these individuals.
Clearly, with younger individuals, we’re mindful of several things. One, the length of time that they have the illness does increase our concern that they have a higher risk of the disease progressing to a more advanced myeloid neoplasm the longer they have the disease. Particularly individuals with 10 years or more of the disease have increasing risk from ET and PV progressing to myelofibrosis. Overall, we think myelofibrosis can be a life-threatening disease, where ET and PV usually can be managed without a decrease in survival. So, that progression is really a negative, and the younger you are, the more exposure you really have to that. Additionally, they have a higher risk of progressing to acute leukemia because of this increased length of time.
Additionally, there are issues as it relates to both the preservation of fertility and the selection of medical therapy. Historically, in ET and PV, there had been a lot of use of the medication hydroxyurea, that is counter indicated in pregnancy, and that has implications in terms of therapy selected, so that medications like interferons or long-acting interferons tend to be preferred in this group of patients, both for that reason, as well as there is the data suggesting that interferons may help to slow the progression of the disease. And again, in younger individuals, that makes it a more relevant therapy for these individuals.