Published on March 30, 2023   50 min

A selection of talks on Clinical Practice

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Hello, my name is Ruben Mesa, and I´m the Executive Director of the Mays Cancer Center at UT Health San Antonio. I'm delighted today to present this update on myelofibrosis: diagnosis, pathogenesis, treatment, and issues with the survivorship of these patients.
Here are my disclosures.
The myeloproliferative neoplasms (MPNs) are a group of chronic leukemias that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocytopenia (ET). They're heterogeneous and they impact the hematopoietic system. Roughly in the United States, we estimate about 350,000 patients, with myelofibrosis being the least prevalent. The presenting symptoms can be somewhat nonspecific, often making the diagnosis challenging.
The natural history of the MPNs begins with earlier disease and earlier disease polycythemia vera, essential thrombocytopenia, and what we call early myelofibrosis, or is now termed pre-fibrotic myelofibrosis. In this phase of these diseases, the time question can be very latent. There can be risky vascular events. There can be symptoms frequently that can be associated with high counts and difficulties with circulation. Now, over time, these diseases can progress to myelofibrosis and this progression is an important transition. We normally feel that patients with ET / PV and early myelofibrosis do not have a dramatically different life expectancy than age-match controls, although there certainly are exceptions in patients with PV and early MF. Those with overt MF have a much more challenging phenotype of the disease. They have progressive constitutional symptoms. They may have cytopenias that can have significant enlargement of the spleen or liver. They may transform into acute leukemia, and the disease can be life-threatening. Roughly the life expectancy in this phase of the disease is three to five years. However, this can be quite heterogeneous. There are some very long-lived individuals. Additionally, many of the therapies which are in development may have an impact that may help to prolong their survival.