Low- to Intermediate-Risk Myelofibrosis: Understanding Disease Progression
Introduction
Myelofibrosis (MF) is a chronic and rare blood cancer that affects the bone marrow’s ability to produce healthy blood cells. Low- to intermediate-risk myelofibrosis is a type of MF that progresses at a slower rate compared to high-risk myelofibrosis.
Understanding Disease Progression in Low- to Intermediate-Risk Myelofibrosis
In the MOST study, the rate of disease progression among patients with low- or intermediate-risk myelofibrosis appeared to increase over time. The criteria for progression included laboratory-defined factors such as low hemoglobin and platelet count, presence of blasts, high white blood cell count, and leukemic transformation. Physician-reported criteria included constitutional symptoms, splenomegaly, red blood cell transfusion, leukemic transformation, and death due to disease progression.
Progression Rates in Cohort A and B
In cohort A, 58.5% of patients experienced disease progression during the study, with hemoglobin below 10 g/dL being the most common criteria. Additionally, 10% of patients died due to disease progression, and 5% had leukemic transformation. In cohort B, 29.6% of patients had disease progression during the course of the study. The median time to meeting criteria for the first and second disease progression in cohort A was approximately two years, while it was approximately one year for reaching the third progression criteria. Importantly, the rate of progression appeared to increase over the course of the study.
Factors Affecting Disease Progression
Looking at patients with and without progression during the study, there was not a significant difference in the median duration of enrollment for patients in cohort A with or without disease progression. However, more patients with progression were receiving ruxolitinib (Jakafi) and fewer were receiving hydroxyurea. Furthermore, most patients in cohort A were receiving MF-directed therapy at the time of enrollment regardless of whether they experienced disease progression.
Treatment for Low- to Intermediate-Risk Myelofibrosis
Combining Anastrozole with Palbociclib, Trastuzumab, and Pertuzumab
Combining anastrozole with palbociclib, trastuzumab, and pertuzumab as a frontline therapy for hormone receptor–positive, HER2-positive breast cancer may avoid some of the toxicities associated with chemotherapy, says Amy Tiersten, MD. However, treatment options for low- to intermediate-risk myelofibrosis depend on the stage and progression of the disease and may include clinical trials, supportive care, or transplant.
Mutation Testing for Treatment Options
Testing for mutations such as JAK2, CALR, and MPL can help determine appropriate treatment options for low- to intermediate-risk myelofibrosis. In cohort A, patients with a JAK2 mutation were more likely to experience disease progression, and similar results were seen for patients with CALR mutations. This information can guide decisions regarding treatment strategies and clinical trials.
Conclusion
Low- to intermediate-risk myelofibrosis is a chronic and rare blood cancer that progresses at a slower rate compared to high-risk myelofibrosis. However, the rate of disease progression can increase over time, and understanding the criteria and factors affecting progression can guide treatment strategies and decisions. Testing for mutations can also provide valuable information for appropriate treatment options.
Originally Post From https://www.cancernetwork.com/view/lower-risk-myelofibrosis-population-has-high-disease-progression-rate
