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Patient Scenario 1: Diagnosis and Risk Stratification of MF
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After reviewing the first patient scenario of myelofibrosis, experts from the John Theurer Cancer Center reflect on best practices in diagnosing and risk stratifying patients.
Transcript:
James K. McCloskey II, MD: Hello, my name is James McCloskey. Welcome to this OncLive® episode of Inside the Clinic. I’m the chief of [the Division of Leukemia] at the John Theurer Cancer Center. And I’m happy to be joined today by some of my colleagues. I’d like them to go ahead and introduce themselves.
Danielle E. Marcotulli, APN: Hi, my name is Danielle Marcotulli. I’m a nurse practitioner at \ John Theurer Cancer Center in the Division of Leukemia.
Geeny Lee, PharmD: Hi, I’m Geeny Lee. I’m an oncology clinical pharmacy specialist at Hackensack University Medical Center and John Theurer Cancer Center.
James K. McCloskey II, MD: Well, let’s go ahead and jump in with our first case. Our first patient is JM. He is a 67-year-old man. And he comes into his primary care physician for his annual checkup … He’s having some fatigue, some bone pain, and these symptoms have been present for the past couple of months. On further questioning, he says that he’s been having some abdominal discomfort as well. He hasn’t noticed a change in his eating habits, but on further questioning, his wife mentioned that she doesn’t feel like he eats as much as he used to. And when we take a look at the EMR [electronic medical records], we notice that he’s actually lost 13 lb since his last visit with us a year ago.
His past medical history is remarkable for a 1-year history of primary myelofibrosis [MF]. He has hyperlipidemia that’s been controlled well with diet and exercise. He’s a nonsmoker but he does drink [alcohol] occasionally … On abdominal exam, he has some palpable splenic enlargement. We note that his spleen is about 4 cm below the left costal margin. And when we look at his labs, his platelets come back at 184. His hemoglobin is 10 [g/dL]. White count is 34,000. And his bone marrow biopsy shows increased megakaryocytes. Molecular testing shows JAK positivity, specifically at V617F. So after discussion with the patient, he [is started] on ruxolitinib, 10 mg twice a day. A year later, he reports back for follow-up. And he tells us that he’s having some increasing fatigue and abdominal pain. When we do labs at this visit his platelets are down to 47,000, his hemoglobin is 8 [g/dL], and his white blood cell count is 29,000. At this time, after discussing options with the patient, we make the switch to pacritinib 200 mg twice a day. Our first question: When we have a patient like JM, how do we go about diagnosing myelofibrosis? What kind of things are we looking for?
Danielle E. Marcotulli, APN: When we’re first seeing them in our office, obviously we’re doing a history and physical, some additional blood work is always helpful, including sometimes next-generation sequencing, which we would often do off a bone marrow biopsy at that point during initial diagnosis. We may also get ultrasound imaging to help us guide in that diagnosis.
James K. McCloskey II, MD: I think that bone marrow biopsy is always an initial part of that evaluation as well. And that’s really important for us next as we … look at the risk stratification of patients. So when we look at this, this is probably one of the most important things that we do in planning their overall treatment and management [of their disease] as a whole. Because it’s really that first snapshot that gives us an idea what to expect, helps us work with our other colleagues in bone marrow transplant. There are a couple of different calculators that we can use. And I think sometimes as a clinician it can be easier to … get lost in them. There are a few things that always jump out as concerning signals, irrespective of which calculator you’re using.
And looking at JM, he has a few of these [concerning signals], particularly cytopenias. Anemia and thrombocytopenia are both predictors of poor outcome. It is important always to take a look at the peripheral blast count in these patients. We obviously recognize the risk of evolution to acute leukemia and myelofibrosis. In many cases leukemogenesis really occurs in … itself. We do keep an eye on that. But those are the things we’re really looking for. Just clinically speaking, in terms of calculators that are available to us, there are a few. I think that most of us, including the calculators that are really used primarily in clinical trials, use DIPSS [Dynamic International Prognostic Scoring System] and DIPSS Plus. These are calculators that primarily take into account the patients’ counts, their symptoms, splenic enlargement. And then really what we’re moving to is a more accurate reflection incorporating molecular data. So that’s where the MIPSS [model] comes in. This incorporates molecular data, including mutations that might occur in ASXL1, EZH2, SRSF2, and IDH1and [IDH]2.
Transcript is AI-generated and edited for clarity and readability.
