The Clinical Management of Primary Myelofibrosis - Episode 2

Presentation and Risk Stratification of Myelofibrosis

Harry Erba, MD, PhD:In looking at her presenting symptoms, physical exam, and blood counts, this is not at all atypical for patients. And in fact, patients like this may be seen by a number of specialists and primary care physicians before they actually get to the hematologist. So, if a blood count isn’t checked and they’re having abdominal fullness, night sweats, pruritus—which is quite common—or fatigue, a number of evaluations for other disorders might have ensued before finally getting to the blood count and showing that the problem is in the bone marrow. So, this is quite typical.

Now the natural history of myelofibrosis is quite varied. Some patients having a very long survival, and some much shorter. And a number of prognostic staging evaluations, or models, have been developed. The first that we’ve been using is Cervantes’ International Prognostic Score criteria, where there are 5 prognostic factors: age over the age of 65, which she has; presence of constitutional symptoms such fevers, night sweats, and weight loss, which she has; anemia with a hemoglobin under 10; leukocytosis with a white-blood cell count over 25,000; and the presence of more than 1% circulating blast. That’s the only one we don’t know that she has, but she has all of the other 4.

If you have none of those factors, then you have low-risk disease with a median survival that exceeds 10 years. However, most patients, 90% of patients, will have intermediate 1—risk disease with 1 risk factor, intermediate-2 with 2 risk factors, or high-risk disease with 3 or more risk factors. The median survival gets progressively worse with each of those. For patients with high-risk disease, the median survival is only about 2 years.

Transcript edited for clarity.

March 2017

  • A 67-year old woman presents to her primary care physician with complaints of fatigue, abdominal fullness, night sweats, and 17-lb weight loss over the past 6 months.
  • PMH includes hypertension, controlled on enalapril 10 mg
  • Abdominal examination reveals spleen palpable 7 cm below the costal margin
  • Lab values:
    • HGB: 9.2 gm/dl
    • Platelets: 189 x 109/L
    • WBC: 19 x 109/L
  • Bone Marrow Biopsy:
    • MF-3
    • CD34+/CD117+ immunostaining demonstrated 1.2% blasts
    • JAK-V617F mutation
  • Diagnosis: Primary myelofibrosis