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What are some of the long-term consequences associated with PV?

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There are a number of long-term consequences of having a diagnosis of PV, which the clinicians need to be cognizant of. The first is thrombotic events, that is the thing that we worry about the most, and we aim our therapy to minimize the risk of having a thrombotic event.

The second issue is disease progression. Now, disease progression can occur in the form of increasing symptoms. These symptoms can include things like night sweats, fevers, weight loss, increase in the spleen size. These can be very debilitating symptoms and certainly can herald the progression of the disease. The disease can also progress in two other forms.

The disease can also progress to the state of myelofibrosis, characterized by splenomegaly, fibrosis in the bone marrow, as well as cytopenias or elevated counts. Finally PV can also progress to acute leukemia.

Acute leukemia that arises from a precursor PV carries a particularly poor prognosis, with most data suggesting that survival is only on the order of two to three months, even with conventional chemotherapy. It’s important that we are cognizant of all of these possibilities at the outset when we’re managing a patient with PV.

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Raajit K Rampal, MD, PhD Hematologic Oncologist Memorial Sloan Kettering Cancer Center | New York, NY
Dr Rampal is a hematology-oncology physician specializing in the treatment of myeloproliferative neoplasms (MPNs) and leukemia. As an active researcher, Dr Rampal is working to understand the genetic events that contribute to the development and progression of MPNs/leukemia and is focused on the development of new and innovative approaches to the treatment of these diseases.