Phlebotomy is typically the first-line treatment for polycythemia vera (PV), and nearly all people with a new diagnosis receive it initially to reduce the risk of complications from the disease.
PV is a form of myeloproliferative neoplasm (MPN) in which the bone marrow makes too many red blood cells. The excess cells clump together, thicken the blood, and slow down its flow, which can cause life-threatening blood clots or bleeding
While there is no cure for PV, there are standard treatment approaches that typically involve the use of baby aspirin, phlebotomy, and medications such as Hydrea (Hydroxyurea), Pegasys (Peginterferon alpha-2a), and Jakafi (Ruxolitinib) that can help manage the symptoms and risks associated with this disease. The goals of treatment are to improve disease-related symptoms, reduce the risk of clot formation and bleeding, and prevent transformation to aggressive cancer.
To learn more about this issue, myMPNteam spoke with Dr. Andrew Kuykendall, a physician who specializes in hematology/oncology and an assistant professor in the department of oncologic sciences at the University of South Florida. He is also an assistant member in the malignant hematology department at the H. Lee Moffitt Cancer Center & Research Institute in Tampa, Florida.
Phlebotomy involves venipuncture, or removing blood from the vein with a medical instrument.
While the procedure, also referred to as bloodletting, may be associated in some people's minds with old-fashioned medicine, people prescribed phlebotomy should not worry. The method has minor side effects, and it is considered the standard of care for people living with PV.
In PV, phlebotomy reduces the number of excess red blood cells by decreasing overall blood volume. While frequency will vary from person to person, people with PV will generally need to have the procedure performed regularly, as red blood cells begin to build up again.
While PV is not curable, it can be managed effectively with phlebotomy. This type of therapy aims to decrease the presence of extra red blood cells, which in turn reduces the risk of blood clots forming inside the blood vessels, a dangerous condition known as thrombosis. Phlebotomy is also aimed at decreasing the severity of PV symptoms.
Phlebotomies are intended to manage PV by lowering hematocrit levels below 45 percent.
After the procedure, the person's blood is thinner, and therefore less likely to cause a phenomenon known as sludging, which occurs when red blood cells gather along the blood vessels' walls.
People with PV who undergo phlebotomy typically find that certain symptoms, such as dizziness, headaches, and ringing in the ears decrease right away.
Phlebotomy is typically appropriate for anybody who has PV, Dr. Kuykendall noted. In fact, he said, treatment with phlebotomy may start as soon as the diagnosis is made.
“Virtually everyone with polycythemia vera will get phlebotomies as part of their treatment plan,” he said. “When people get diagnosed, we're talking about hematocrits that are often in the 55 percent to 60 percent range, sometimes a little bit higher, and our goal is to keep that below 45 percent at least.”
The goal of administering phlebotomy to someone with PV is to both treat symptoms related to the disease and to prevent known complications of the disease — namely, blood clots, Dr. Kuykendall said. These can range from an embolism (a blood clot that travels from one part of the body to another, such as the lung) to a stroke or a heart attack.
“Our goal is twofold, and maybe threefold, in treating these diseases,” he said. “One — we want to prevent thrombotic events because we know that's a big problem. Two — we want to maximize quality of life, and then, obviously, we would like to prevent disease progression.”
Dr. Kuykendall said that in many cases, people with PV who have their first phlebotomy report improvements in their symptoms in reasonably short order. Headaches are a common symptom of PV, but those tend to decrease in intensity after phlebotomy, he said.
"Often with PV, it's this heaviness, this fullness, this pressure-like headache," Dr. Kuykendall noted. "They'll talk about those headaches [improving] after each phlebotomy ... treating a disease leads to the resolution of the symptom, so that's helpful there."
Phlebotomy is not a one-and-done proposition, so people with PV will typically need to have the procedure performed on an ongoing basis. The frequency of the treatment depends on the person receiving it.
“Ninety percent of the time, these patients are getting diagnosed and starting on a pretty aggressive phlebotomy course in the early onset, maybe a weekly phlebotomy for four weeks and then being able to back off as tolerated,” Dr. Kuykendall said. “At our institution, we have a protocol that we put in place that puts people on weekly phlebotomies until they no longer require it, and then we start to back off.”
Generally speaking, phlebotomies do not come with significant side effects, although Dr. Kuykendall did say that there is a risk of iron deficiency.
“Within polycythemia vera, what we often run into is patients who get phlebotomy after phlebotomy after phlebotomy, and get iron-deficient from that,” he said. “They start suffering from side effects of being iron-deficient, and then if we give them iron back, that gives them fuel to make more red blood cells, and so then they start to need more phlebotomy again. You can run into this nasty circle.”
Some lightheadedness and dizziness after phlebotomy are not uncommon. To prevent this, stay hydrated by drinking about eight glasses of water the day prior to the procedure. After the procedure, continue to drink the same amount of fluids for a day or two. Avoid alcohol, tobacco, and caffeine for the rest of the day after the procedure and stay away from strenuous exercise, such as jogging, for at least one day afterward.
On myMPNteam, you can connect with other people who have MPNs and polycythemia vera. More than 800 people come together on myMPNteam to ask questions, give advice, and share their stories with others who understand life with MPNs.
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