Anemia is a common and potentially dangerous condition that can occur in patients with myelofibrosis, a type of myeloproliferative neoplasm. While anemia is a blanket term that describes low hemoglobin levels, myelofibrosis-related anemia behaves quite differently than anemia in patients with a blood cancer diagnosis, explained Sharon Bledsoe.
Bledsoe, a senior research nurse at The University of Texas MD Anderson Cancer Center in Houston, recently explained myelofibrosis-related anemia, including its cause and treatment.
CURE®: What causes anemia in patients with myelofibrosis?
Myelofibrosis is basically a disease in which the bone marrow gets replaced by connective tissue in a process called fibrosis. The bone marrow’s main objective is to produce blood cells, and in producing the blood cells — the red blood cells, the white blood cells and the platelets. When the fibrosis interferes with the production of the cells, scar tissue starts to form in the bone marrow, and the bone marrow is the soft spongy tissue in the center of the bones.
As the scar tissue starts to grow, the bone marrow loses its ability to make enough healthy blood cells. So, it produces too many abnormal blood cells. The lifespan of a true red blood cell or of a normal human red blood cell is 120 days, which is about roughly four months. When you have a patient dealing with myelofibrosis, with the scarring and all of that, they’re producing the red blood cells, but they’re not maturing; when they’re not mature, they die off faster. So, they’re producing a whole lot a whole lot, but they’re dying fast. Then you have the anemia that starts because they’re not living for enough time, so they’re not getting four months of life; within days, weeks, they’re dying off.
How is myelofibrosis-associated anemia treated?
When patients’ (hemoglobin levels) start to get low, we start to monitor their trends. We monitor their hemoglobin; we start to monitor in to see if they’re if they are going to need a transfusion. And if they need transfusions, how often are they needing the transfusions? So we’re going to monitor all of that, whether they need the transfusions and how often they need the transfusions.
That’s one way that it’s treated.
And then doctors may put them on drugs that will help the anemia, drugs like danazol and Jakafi (ruxolitinib). When those red blood cells are being produced so quickly, there’s not enough room within the bone marrow, so (they) go into the spleen, or into the liver, which is now causing them to have enlarged spleens and enlarged liver. And sometimes, because it can’t be treated, the spleen has to be removed.
(Jakafi) can take the spleen size down. However, with (Jakafi), you fall into the area where they can get skin cancers, secondary skin cancers, squamous cell carcinoma, basal cell carcinoma and things like that; you have to really, really watch for that. In treating the anemia, you have to watch for so many other things that may crop up.
What is the difference between myelofibrosis-related anemia and general anemia that is experienced in patients without an MPN?
There is a major difference, because anemia that a person would have that doesn’t have cancer presents differently than the anemia (related to MPNs). For a person who has a blood cancer, their anemia is going to come with other things (such as) possibly filling up faster (when eating), night sweats (and) extreme fatigue. And some of them have (feelings of) wanting to faint because the hemoglobin is so low.
In a person that has just routine anemia, they won’t have those types of symptoms, they’ll just probably feel a little tired or a little sluggish.
What advice do you have for patients with myelofibrosis who may be experiencing anemia?
We tell the patients to let us know if you have increased fatigue, let us know if any of your symptoms change, you have increased fatigue, you have increased night sweats, you start having fevers or things like that, let us know if any of those things are taking place. That way, I can give that information to the to the oncologist and then they know what to do for the patient.
Make sure that you are proactive as a patient, if there’s something that’s wrong, and you know that it hasn’t been an issue before, make sure that you follow up and follow through, don’t just accept (symptoms). If you have to go to 5 doctors, go to as many as it takes to get the diagnosis, because with time, time loss is not time that can be regained. So, you need to be proactive and monitor your care and know what your norms are and what’s not normal for you.
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