What is involved in determining low risk versus high risk myelofibrosis? Expert Dr. Michael Grunwald from Levine Cancer Institute discusses IPSS, DIPSS, MYSEC-PM, and MIPSS70 scoring systems, key patient factors they weigh in determining risk, and why risk stratification is an essential part of myelofibrosis care. 

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“…risk stratification is important because it can impact treatment choices including whether to initiate treatment, whether to pursue transplantation, and sometimes the type of treatment as well. Also, lower risk patients can require treatment at times for symptoms and splenomegaly in myelofibrosis.”

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Transcript:

Lisa Hatfield:

Dr. Grunwald, what is lower risk versus higher risk myelofibrosis?

Dr. Michael Grunwald:

Fortunately, we have various risk stratification systems available. We have the International Prognostic Scoring System or IPSS, and then later on, the Dynamic International Prognostic Scoring System or DIPSS scoring system was developed to risk stratify patients. And then the DIPSS Plus, there’s the MYSEC-PM scoring system, which is specifically for patients who have a history of essential thrombocythemia or ET, or polycythemia vera or PV, who then developed myelofibrosis.

And then finally, we have the Molecular Scoring Systems, the Molecular International Prognostic Scoring System, which is called the MIPSS70. And then the newest one of those is the MIPSS70 Plus version 2.0. So we have a lot of different risk stratification systems and they have many features in common.

These risk stratification systems look at patient’s age, their blood counts, sometimes whether patients are so anemic that they’re requiring blood transfusions, the percentage of blasts in the peripheral blood, the degree of fibrosis or scar tissue in the bone marrow, their cytogenetics. So whether patients are missing big chunks of genes in their bone marrow or whether chunks of genes are translocated from one chromosome to another chromosome.

And then finally, the Molecular Scoring Systems take into account individual genetic mutations. Based on these features of a patient’s disease, we can determine whether a patient is at high or low risk of progression and also high or low risk of mortality from myelofibrosis. And the scoring systems are all a little bit different, so it’s confusing, but there are certain features that are common among low risk patients. So more normal blood counts, lower percentages of blasts in the peripheral blood, less fibrosis in the bone marrow, and then more favorable mutations, which could be chromosomal abnormalities or individual genetic mutations. Higher risk patients tend to have more abnormal blood counts, higher blast percentages, more fibrosis in the marrow, and then unfavorable risk mutations.

And from this we get a sense of whether a patient’s disease is likely to progress to acute leukemia, and also whether a patient is at risk of death from myelofibrosis in the near future. This information can be very helpful because it can guide us in our recommendations for treatment or sometimes for no treatment for a patient.

Lisa Hatfield:

Ok, thank you. So if I were your patient and I was just recently diagnosed with myelofibrosis and I said I heard that I was staged or given the risk stratification from the DIPSS system, would you know what that means and how that might relate to other systems of staging or do I need to be aware myself that it means this, that it means I am lower risk or higher risk, will you tell me that if I was sitting in your office?

Dr. Michael Grunwald:

Yes. So I went through this with patients yesterday where we sat together and we looked at the scoring systems, and we looked at a few of the scoring systems, and we plugged in patients’ numbers into scoring system calculators that are available online. So I can plug in the white count, I can plug in the patient’s platelet count, their hemoglobin, their mutations, and figure out what their DIPSS score is, what their DIPSS Plus score is, what their MIPSS70 score is. And I like it when the information from the different scoring systems is fairly concordant.

For example, if a patient is low risk by all of the risk stratification systems, makes me very confident that a patient is low risk. And then if there’s more discordance where, let’s say, a patient has a molecular mutation that indicates high risk and heavily sways the MIPSS70 or MIPSS70 Plus version 2.0 toward the higher end of the risk spectrum, and we have another scoring system, one of the older ones that would indicate lower risk, that’s where the conversation is a little bit more difficult. And I tend to trust the newer molecular systems a little more, especially in patients who had no previous history of essential thrombocythemia (ET) or polycythemia vera (PV). And we do discuss that with the patients, both the clinical attributes and the genetic attributes of the disease.

My [ACT]IVATION tip for this question is, risk stratification is important because it can impact treatment choices including whether to initiate treatment, whether to pursue transplantation, and sometimes the type of treatment as well. Also, lower risk patients can require treatment at times for symptoms and splenomegaly in myelofibrosis.

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