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Understanding Myelofibrosis with Dr. John Mascarenhas
Dr. John Mascarenhas, a hematologist oncologist, explains what myelofibrosis is, how it can present and how it is diagnosed.
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Understanding Myelofibrosis with Dr. John Mascarenhas
What is Myelofibrosis?
DR. JOHN MASCARENHAS:
Myelofibrosis is a blood cancer.
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Dr. John Mascarenhas
Hematologist Oncologist
DR. JOHN MASCARENHAS:
“So myelofibrosis is a blood cancer. It comes from the bone marrow. It results in fibrosis or scarring in the bone marrow, and often low blood counts that can really impact patients, so patients often do not feel well. They are going to have a lot of different symptoms, and it can really be debilitating. Unfortunately, it's a chronic disease, meaning it doesn't spontaneously remit. It is eventually progressive. It can progress in different ways for different patients. I'm not sure there's a typical presentation, actually. The presentations can be wide and varied. I really think it's very heterogeneous and variable the way patients present in their clinical course.”
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What are the signs and symptoms of myelofibrosis?
DR. JOHN MASCARENHAS:
“So sometimes you see patients present to a primary physician with complaints that might be protean, systemic symptoms like fevers, night sweats, weight loss. Then the workup ensues, and perhaps blood counts are abnormal. That then triggers a referral to a hematologist who then ultimately does a bone marrow biopsy and the diagnosis is made.”
“Sometimes patients end up in the emergency room, sometimes for clotting reasons, bleeding reasons, or acute issues, and then the diagnosis may be ultimately made. Then sometimes patients really are misdiagnosed or not correctly diagnosed and given a different diagnosis, perhaps like myelodysplastic syndromes or other diseases that can masquerade or be mimics of myelofibrosis, and I've seen that happen until it finally is confirmed.”
“So it really can be quite different. I don't really think any two patients really present in the exact same way and have the same course, and patients come through the door through various mechanisms, either through referrals from primary physicians, diagnosis sometimes within the hospital. Increasingly patient referrals, patients are advocates for themselves and their families are often advocates for themselves, and they will find centers of excellence because the reality is myelofibrosis and myeloproliferative neoplasms are rare diseases.”
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How is myelofibrosis diagnosed?
DR. JOHN MASCARENHAS:
“Myelofibrosis itself has to be diagnosed with a bone marrow biopsy, nowadays with mutational testing, chromosomal testing, and putting all of that together so that you meet certain World Health Organization criteria for either primary myelofibrosis or secondary myelofibrosis from an antecedent essential thrombocythemia or polycythemia vera.”
“So, getting the diagnosis is crucial because the treatments are tailored to the diagnosis and the subtypes of disease. Then I think making sure that your disease or the treatment plan for your disease is personalized and tailored to the goals of therapy for you because it really is, as I keep saying, it's variable. It's variable in how patients present their clinical course, and their treatment goals could be different.”
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MMLCOCO230009 May 2023
Produced in USA.