Patients should understand the different prognostic scoring systems used in myelofibrosis, and how the results can impact treatment decisions.
Since the majority of treatments for myelofibrosis are palliative – meaning that they focus on treating symptoms, not the disease – scoring systems that help predict patients’ prognoses are particularly important, explained Dr. Ellen L. Ritchie, assistant professor of medicine and a member of the Leukemia Program at the Weill Cornell Medical College of Cornell University and the New York Presbyterian Hospital.
With transplant being the only curative treatment for the disease, patients and clinicians need to understand what other factors can tell them more about their myelofibrosis outcomes, according to Ritchie’s presentation at the 39th Annual CFS® Meeting.
“When you have a disease with very few effective treatments, there is an abundance of scoring systems that are developed to assign prognosis,” Ritchie said. “And these scoring systems are meant to help guide us whether or not we should send a patient to stem cell transplant.”
IPSS and DIPSS
At diagnosis, a patient is likely to be scored using the International Prognostic Scoring System (IPSS), then, at any point during the patient’s disease trajectory, they might be scored using the Dynamic IPSS (DIPSS), which looks at the following, giving a stronger weight to hemoglobin levels, according to Ritchie:
Another common scoring system that helps clinicians determine a patient’s prognosis is the Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients with Myelofibrosis – commonly referred to as MIPSS70. This system has different age categories than the DIPSS and looks at whether or not there is bone marrow fibrosis, as well as genetic mutations. A second version of the scale includes patient sex, severity-adjusted hemoglobin levels, karyotype (a patient’s individual chromosomes) and genes.
“It stratifies (patients) from a lower-risk to a very high-risk karyotype,” Ritchie said. “It also stratifies whether there is more than one high-risk mutation. These patients are stratified into very low-risk to very high-risk levels, and that allows you to make maybe a discriminatory decision.”
A third type of prognostic scoring system for patients with myelofibrosis is the Genetically Inspired Prognostic Scoring System for Primary Myelofibrosis (GIPSS), which determines if a patient’s karyotype is favorable or unfavorable solely based on genetic data.
Ritchie said that this data is usually easier to decipher and use for treatment decisions, though it can take a long time to get the genetic data back.
“In the myelofibrosis community, we’re working really hard to try to integrate molecular data into our scoring systems,” she said.
While none of the prognostic scoring systems are perfect, it is important for patients to discuss the results with their clinicians, as they can have implications on next steps for treatments. For example, patients who are deemed as low-risk can opt for a watch-and-wait approach, while those with intermediate risk might want to look into trials or transplantation.
“The scoring systems are meant to help guide us whether or not to send a patient to stem cell transplant,” Richtie said.
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