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MPN Workshop of the Carolinas 2024 | The current approach to managing patients with prefibrotic PMF
Prithviraj Bose, MD, The University of Texas MD Anderson Cancer Center, Houston, TX, discusses the current strategies for the management of patients with prefibrotic primary myelofibrosis (PMF). As in essential thrombocythemia (ET), the short-term complications of patients with prefibrotic PMF are vascular; therefore, the approach to their management and risk stratification resembles that utilized in patients with ET. This interview took place at the 1st Annual MPN Workshop of the Carolinas in Asheville, NC.
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Transcript
Prefibrotic primary myelofibrosis patients are generally approached as if they have ET, even though we know that this is a worse disease than ET prognostically, but because their short term problems are vascular, like in ET, some of the same principles are applied to them. And in fact, it’s been shown that the IPSET, which is a way of stratifying risk stratifying ET patients for risk of thrombosis, can be used in in prefibrotic primary myelofibrosis...
Prefibrotic primary myelofibrosis patients are generally approached as if they have ET, even though we know that this is a worse disease than ET prognostically, but because their short term problems are vascular, like in ET, some of the same principles are applied to them. And in fact, it’s been shown that the IPSET, which is a way of stratifying risk stratifying ET patients for risk of thrombosis, can be used in in prefibrotic primary myelofibrosis. So that’s been shown.
But at the same time they are not ET, so they are actually an early form of myelofibrosis. So, this is where it gets a little bit murky but also more interesting in the sense can we intervene early and prevent the adverse outcomes that we see with our overt primary myelofibrosis patients. And while these studies were not strictly done in just prefibrotic, because that requires a pathologic definition, with that caveat, I will say that there is a lot of evidence mounting for using ruxolitinib early and interferons in these settings. So it could be prefibrotic by pathology or it could be just clinically early myelofibrosis.
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Disclosures
Research support: Incyte, BMS, CTI (Sobi), Morphosys, Karyopharm, Sumitomo, Ajax, Disc, Ionis, Geron, Janssen, Cogent, Blueprint, Kartos, Telios.; Honoraria/consulting fees: Incyte, BMS, CTI (Sobi), GSK, Abbvie, Morphosys, Pharma Essentia, Sumitomo, Karyopharm, Ionis, Disc, Geron, Morphic, Jubilant, Ono, Novartis, Blueprint, Cogent.
