Polycythemia vera

Since its discovery, polycythemia vera (PV) has challenged clinicians responsible for its diagnosis and management and scientists investigating its pathogenesis. As a clonal hematopoietic stem cell (HSC) disorder, PV is a neoplasm but its driver mutations result in overproduction of morphologically and functionally normal blood cells. PV arises in an HSC but it can present initially as isolated erythrocytosis, leukocytosis, thrombocytosis, or any combination of these together with splenomegaly or myelofibrosis, and it can take years for a true panmyelopathy to appear. PV shares the same JAK2 mutation as essential thrombocytosis and primary myelofibrosis, but erythrocytosis only occurs in PV. However, unlike secondary causes of erythrocytosis, in PV, the plasma volume is frequently expanded, masking the erythrocytosis and making diagnosis difficult if this essential fact is ignored. PV is not a monolithic disorder: female patients deregulate fewer genes and clinically behave differently than their male counterparts, while some PV patients are genetically predisposed to an aggressive clinical course. Nevertheless, based on what we have learned over the past century, most PV patients can lead long and productive lives. In this review, using clinical examples, I describe how I diagnose and manage PV in an evidence-based manner without relying on chemotherapy. Polycythemia vera (PV) is the commonest myeloproliferative neoplasm (MPN), the ultimate phenotypic consequence of JAK2 ...

What is polycythemia vera? Polycythemia vera is a rare blood disorder in which there is an increase in all blood cells, particularly red blood cells. The increase in blood cells makes your blood thicker. This can lead to strokes or tissue and organ damage. What causes polycythemia vera? Polycythemia vera is caused by a genetic change (mutation) that develops during your lifetime. It is not an inherited genetic disorder. In most cases it is not known why this happens. What are the symptoms of polycythemia vera? When you have more blood and it is thicker than normal, problems can occur. Each person’s symptoms may vary. Symptoms may include: • Lack of energy (fatigue) or weakness • Headache • Dizziness • Shortness of breath and trouble breathing while lying down • Vision problems, such as double vision, blurred vision, and blind spots • Inability to concentrate • Night sweats • Face and becomes red and warm (flushed) • Nosebleeds • Bleeding gums • Too much menstrual bleeding • Coughing up blood • Bruising • Itchy skin (often after a hot bath) • Gout • Numbness • High blood pressure These symptoms may look like other blood disorders or health problems. Always see your healthcare provider for a diagnosis. How is polycythemia vera diagnosed? Your healthcare provider will take your medical history and give you a physical exam. Your provider may also do blood tests. These tests will check the increased number of red blood cells in your body. They will also check if there are other...

Polycythemia vera (PV) causes your bone marrow to make too many red blood cells. You need these cells to carry oxygen around your body, but too many of them can make your blood thicken and form clots. Sometimes blood clots lead to a heart attack or stroke. Treatments lower the number of red blood cells and prevent blood clots. They also relieve other symptoms, like headaches and vision problems. With the right treatment, you can stay healthy -- and feel better. Phlebotomy This is the main PV treatment. It removes some of your blood to get rid of extra red blood cells. It also lowers your red blood cell count, so your blood thickness starts to get closer to normal. You may get phlebotomy when your doctor first tells you that you have PV. It's a lot like donating blood. The doctor or nurse will put a needle into a vein in your arm and remove a small amount of blood from your body. The goal is to lower your hematocrit level. Hematocrit is the percentage of red blood cells compared with the total amount of blood. You'll get this treatment once a week or month until your hematocrit goes down to around 45%. Once your red blood cell level has dropped, you’ll get phlebotomy less often. Medicines After your red blood cell level drops, you'll take one of these prescription drugs to slow the production of new red blood cells in your bone marrow. Hydroxyurea is a cancer drug that slows down the growth of new cells in your body. In PV, it lowers the number of red blood cells and platel...

It’s important for you to know that the dietary modifications you need to follow with polycythemia vera are safe and are not extremely difficult to manage. You will only have to reduce your intake of a few types of foods. While the recommendations aren’t highly restrictive, the dietary habits you need to follow when you have polycythemia vera require planning and attention. Over time, you will learn to adjust to these modifications, and they can become healthy habits for you. What to Eat Low energy can be a problem with polycythemia vera. So, you can’t skimp on calories. The best way to maintain your energy is by avoiding simple sugars and by eating foods that contain complex carbohydrates (like vegetables and whole grains) and lean proteins. These will provide you with lasting energy throughout the day. • Water: Polycythemia vera causes excess red blood cells to circulate in your blood vessels and increases the risk of kidney stones and gout. Drinking enough water is vital to address these issues. Getting ample amounts of water won’t completely solve these problems, but it can help reduce their severity and risk. • Calcium: Polycythemia vera is associated with bone marrow damage, which can increase your risk of bone fractures, making it important to get enough calcium in your diet. This mineral helps build and maintain bone strength. • Oxalate: You can also develop kidney stones, a condition that causes flank pain (pain in the lower back on one side), and possibly blood i...

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Polycythemia vera (PV) is a rare type of blood cancer in which your body produces too many red blood cells. Red blood cells carry oxygen throughout your body. When you have too many red blood cells, your blood thickens and flows more slowly. The red blood cells can clump together and form clots inside your blood vessels. If it’s not treated, PV can lead to life threatening complications. Blood that flows more slowly can reduce the amount of oxygen that reaches your heart, brain, and other vital organs. And blood clots can completely block blood flow within a blood vessel, causing a stroke or even death. In the long term, PV can lead to scarring of the bone marrow, known as There’s no cure for PV, but you can manage the condition with treatment. Your doctor will likely do routine blood draws and prescribe medication to help prevent serious blood clots. It’s important to talk with your doctor if you’re at risk of PV and have any of its symptoms. PV might not cause any symptoms for many years. When symptoms first start, they can be mild enough to miss. You might not realize you have PV until a routine blood test picks up the problem. Recognizing the symptoms early can help you get treatment started and hopefully prevent blood clots and their complications. Common symptoms of PV include: • fatigue • itching • trouble breathing when you lie down • trouble concentrating • unintended weight loss • pain in your abdomen • feeling full easily • blurred or double vision • dizziness •...

There are three main blood tests that are used to diagnose PV: Complete blood count (CBC) A Hemoglobin is a protein rich in iron that helps red blood cells carry oxygen from the lungs to the rest of the body. And if you have PV, your hemoglobin level will be elevated. Usually, the more red blood cells you have, the higher your hemoglobin level. In adults, a hemoglobin level greater than 16.0 grams per deciliter (g/dL) in women or 16.5 g/dL in men can indicate PV. A CBC will also measure your hematocrit. Hematocrit is the volume of your blood that’s made up of red blood cells. If you have PV, a higher-than-normal percentage of your blood will be made of red blood cells. In adults, a hematocrit greater than 48 percent in women or greater than 49 percent in men can indicate PV, according to the Blood smear A blood smear looks at your blood sample under a microscope. This can show the size, shape, and condition of your blood cells. It can detect abnormal red and white blood cells, along with platelets, that can be linked to Erythropoietin test Using a blood sample, an erythropoietin test measures the amount of the hormone erythropoietin (EPO) in your blood. EPO is made by the cells in your kidneys and signals stem cells in the bone marrow to make more red blood cells. If you have PV, your EPO level should be low. This is because your EPO isn’t driving blood cell production. Instead, a JAK2 gene mutation is driving blood cell production. Bone marrow tests can determine if your ...