Ruben A. Mesa, MD, FACP:This particular patient, if we use the DIPSS-plus [dynamic international prognostic scoring system plus] criteria, which is probably the most commonly used at the current time, would have several risk factors that would weigh in: Their age, being over age 65; the presence of anemia, a hemoglobin of less than 10; the presence of symptoms; and the presence of greater than 1% blasts. This individual would fall into the category of being intermediate-2 by that risk set of criteria.
Features that the patient did not have that would be additional factors that would make someone high risk would include transfusion dependence, a high-risk karyotypethis individual had a mutation in the 20th chromosome, which is not a high-risk mutation—or the presence of thrombocytopenia.
There are multiple classification systems and prognostic scores for myelofibrosis. Which one you can utilize is in part dependent on which of the variables you have available to you. Through the DIPSS, you can calculate by your physical exam, your symptom assessment, and a complete blood count. With the DIPSS-plus, we were able to calculate this patient because we also had the chromosomal analysis available, and this individual did not have a high-risk chromosome analysis.
The MIPSS70 [mutation-enhanced international prognostic scoring system for transplant-age patients] is for individuals under the age of 70. It is meant to inform for individuals who might be a candidate for stem cell transplantation. One requires the next-generation sequencing results to look for the presence of high-risk molecular features in such an individual. So we’d not be able to utilize it in this patient.
In many patients with myelofibrosis, we are unable to obtain an accurate karyotype due to the fibrosis that occurs in the marrow. When we are able to obtain a karyotype, many of the prognostic data that have arisen are very consistent with what we have seen with other chronic myeloid disorders, including myelodysplastic syndrome and acute myeloid leukemia. In particular, complex chromosomal changes, meaning multiple abnormalities, deletions of chromosome 5, 7, etcetera, are all adverse, or high risk. It is probably the minority of patients with myelofibrosis who have very adverse chromosomal abnormalities. However, they do tend to accumulate more chromosomal abnormality as they progress toward acute myeloid leukemia. So that issue of clonal progression is reflected in the chromosomal abnormalities.
Prognostic factors tell us how long we might expect an individual myelofibrosis patient to live. Now, many of these criteria from the past were generated based on retrospective data in patients with myelofibrosis that preceded the JAK inhibitor era. We are not quite certain as to the relevance of these prognostic features and how they relate to our current medical therapies and outcomes.
It is not clear that there is a specific chromosomal change linked with the outcomes associated with JAK inhibitor therapy at this time. That may evolve in the future, but at the current time, it is not a major component of our processes. I view that these prognostic scores are the most beneficial in helping us estimate survival and make decisions as they relate to the timing of stem cell transplantation.
Transcript edited for clarity.
Case: 72-Year-Old Man Diagnosed With Primary Myelofibrosis
December 2018
Connecting Spleen Volume Reduction to Survival Outcomes in MF
April 21st 2024During a Case-Based Roundtable® event, Raajit K. Rampal, MD, PhD, discussed the correlation between spleen volume responses and survival outcomes for patients with myelofibrosis in the second article of a 2-part series.
Read More
Savona Discusses First-Line JAK Inhibition for Patients With Myelofibrosis at Risk of Anemia
April 17th 2024During a Case-Based Roundtable® event, Michael Savona, MD, and participants discussed the case of a patient with myelofibrosis and moderate anemia receiving JAK inhibitor therapy.
Read More
PTCy Offers New Hope for Mismatched Stem Cell Transplants in Leukemia, MDS
April 13th 2024Jeff Auletta, MD, discussed how PTCy-based graft-vs-host disease prophylaxis offers a promising approach for expanding access to successful cell transplantation regardless of donor match or patient ethnicity.
Read More
Scott Evaluates Treatment Options for Hydroxyurea-Resistant Polycythemia Vera
March 28th 2024In a Community Case Forum event in partnership with the Washington State Medical Oncology Society, Bart Scott, MD, broke down various trials of hydroxyurea, ruxolitinib, and interferon in patients with polycythemia vera to assess outcomes such as hematocrit control and molecular response.
Read More