Polycythemia Vera

Polycythemia vera is a rare type of blood cancer. This means there's a problem with the bone marrow, the part of your body that makes blood cells. In polycythemia vera, the bone marrow produces too many red blood cells. Having extra red blood cells makes your blood thicker than normal. This thicker blood flows more slowly, which can lead to serious problems, like blood clots.

These blood clots can form in different parts of the body and cause various issues. Think of it like trying to pour syrup through a straw – it's much harder and slower. Similarly, slow-moving blood can cause issues with blood flow to the heart, brain, or other organs.

Polycythemia vera often develops gradually. This means you might have the condition for a while without noticing any symptoms. Sometimes, people discover they have it during a routine blood test for something completely different.

If polycythemia vera isn't treated, it can be very dangerous. Early diagnosis and appropriate medical care are important. Treatment can help manage the symptoms, prevent complications, and improve your overall health and well-being.

Polycythemia vera can sometimes have no noticeable symptoms at all. For others, the early signs might be very general, like a headache, feeling lightheaded, tiredness, or seeing things less clearly.

However, some people experience more specific symptoms. These include:

• Itchiness: This often gets worse after taking a hot bath or shower. It's a common complaint, and the intense itchiness can be quite bothersome.
• Numbness, tingling, burning, or weakness: These sensations can affect the hands, feet, arms, and legs. They may feel like pins and needles or a persistent discomfort.
• Feeling full quickly after eating, bloating, and/or pain in your upper left belly: This happens because the spleen, a part of your immune system, can become enlarged. The enlargement puts pressure on the area and causes these uncomfortable sensations.
• Unusual bleeding: This might be a nosebleed, bleeding gums, or other bleeding that's not typical for you.
• Joint pain and swelling: Some people experience pain and swelling in a joint, often the big toe. This can be a significant discomfort.
• Shortness of breath and trouble breathing when lying down: This symptom can be caused by the increased blood volume associated with polycythemia vera. It's particularly noticeable when resting in a horizontal position.

If you notice any signs or symptoms that might be related to polycythemia vera, it's important to schedule a doctor's appointment. Polycythemia vera is a condition where your body produces too many red blood cells. These extra red blood cells can thicken your blood, potentially leading to health problems. Symptoms can vary but might include headaches, dizziness, itching, or fatigue. Seeing a doctor is the best way to get a proper diagnosis and appropriate treatment, if needed.

Polycythemia vera is a condition where a change (mutation) in a gene affects how your body makes blood cells. Your body usually carefully controls the number of red blood cells, white blood cells, and platelets. But in polycythemia vera, the bone marrow, the part of your body that makes blood cells, starts producing too many of certain types of blood cells.

Scientists don't know exactly what causes this gene change, and it's not typically passed down from parents to children.

Polycythemia vera is a condition that can affect people of any age, but it's most often diagnosed in adults between 50 and 75 years old. While men are diagnosed with it more frequently, women can develop it, sometimes at younger ages. In short, this blood disorder can happen to anyone, although certain age groups and genders are slightly more prone to developing it.

Polycythemia vera can lead to several problems. Here are some of the potential complications:

Blood Clots: Polycythemia vera causes the blood to thicken and flow more slowly. This, combined with changes in the blood's platelets, increases your chance of developing blood clots. These clots can travel to different parts of the body, potentially causing a stroke, heart attack, or blockage in your lungs (pulmonary embolism), a leg vein (deep vein thrombosis), or a vein in your abdomen.

Enlarged Spleen: Your spleen helps your body fight off infections and get rid of old or damaged blood cells. When you have polycythemia vera, your body produces too many blood cells. This extra work puts a strain on your spleen, making it swell up.

Problems from Too Many Red Blood Cells: Having too many red blood cells can cause other issues. For example, it can lead to sores in the lining of your stomach, upper small intestine, or esophagus (peptic ulcers). It can also cause inflammation in your joints, a condition called gout.

Other Blood Disorders: In some rare cases, polycythemia vera can progress to other blood disorders. These can include a condition where scar tissue replaces your bone marrow, problems with stem cells not developing properly, or even blood and bone marrow cancer (acute leukemia). It's important to remember that these are not common outcomes, but they are possible complications of polycythemia vera.

To diagnose polycythemia vera, your doctor will first ask about your medical history and do a physical exam. They'll look for signs and symptoms that might suggest the condition.

If your doctor thinks you might have polycythemia vera, they'll likely order some blood tests. These tests can show if you have:

• More red blood cells than usual: Your body might be making too many red blood cells, which can lead to an increase in blood volume and thickness. Sometimes, there are also higher-than-normal levels of platelets (cell fragments that help blood clot) or white blood cells (part of your immune system).
• A higher percentage of red blood cells in your blood: This is measured by a test called a hematocrit. A higher hematocrit means your blood has a greater proportion of red blood cells, making it thicker.
• High levels of hemoglobin: Hemoglobin is a protein in red blood cells that carries oxygen throughout your body. Higher levels of hemoglobin can indicate an increase in red blood cells.

To confirm the diagnosis, your doctor might recommend a bone marrow test. This test involves taking a small sample of your bone marrow. Bone marrow is the soft, spongy tissue inside your bones, where blood cells are made.

There are two main types of bone marrow tests:

• Bone marrow aspiration: A thin needle is used to remove a small amount of the liquid part of your bone marrow, usually from your hip bone.
• Bone marrow biopsy: At the same time as the aspiration, a small piece of bone tissue and the marrow within it is also removed.

If you have polycythemia vera, the analysis of your bone marrow or blood sample might reveal a specific gene mutation associated with the disease. This mutation can help confirm the diagnosis.

Polycythemia vera has no cure. Instead, treatments focus on preventing problems it can cause and easing symptoms.

A common treatment is phlebotomy, where blood is drawn regularly from a vein. This is similar to donating blood. Phlebotomy reduces the amount of blood and the excess blood cells. How often this needs to be done depends on how severe the condition is.

If itching is a problem, doctors might prescribe antihistamines or recommend ultraviolet (UV) light therapy. In some cases, medications typically used for depression, called selective serotonin reuptake inhibitors (SSRIs), have helped with itching. Examples of SSRIs include paroxetine (brand names include Brisdelle, Paxil, Pexeva) and fluoxetine (brand names include Prozac, Sarafem, Selfemra).

If just drawing blood isn't enough, other medicines can help reduce the number of red blood cells in the body. These medications include:

• Hydroxyurea (Droxia, Hydrea): This medicine can help control the production of red blood cells.
• Interferon alfa-2b (Intron A): Another medicine that can reduce red blood cell production.
• Ruxolitinib (Jakafi): This medication can help manage the disease.
• Busulfan (Busulfex, Myleran): This medicine is used in some cases to reduce the number of blood cells.

Doctors will also likely prescribe medicines to manage other health risks that can occur with polycythemia vera. These might include high blood pressure, diabetes, and high cholesterol. A low dose of aspirin may also be recommended to lower the risk of blood clots and can sometimes help with burning pain in the hands or feet.

Taking care of yourself is important if you have polycythemia vera. Here are some helpful tips:

Skin Care: Itching is a common problem with polycythemia vera. To help with itching, try these steps:

• Cool Baths: Take cool baths or showers, not hot ones. Using cool water can soothe your skin.
• Gentle Cleansing: Use a mild, gentle soap or cleanser.
• Patting Dry: Gently pat your skin dry instead of rubbing it.
• Starch Baths: Adding a small amount of cornstarch to your bath might help reduce itching.
• Avoid Irritants: Stay away from hot tubs, heated whirlpools, and very hot showers or baths.
• Don't Scratch: Itching can be tempting, but scratching can damage your skin and increase the risk of infection. Instead, use moisturizing lotion to keep your skin soft and healthy.

Lifestyle Choices:

• Exercise Regularly: Moderate exercise, like walking, is good for your overall health. It can also improve blood flow and lower your risk of blood clots. Gentle stretches for your legs and ankles can also help improve circulation.
• Quit Smoking/Tobacco Use: Smoking narrows your blood vessels, which increases the risk of blood clots and problems like heart attacks and strokes. Quitting is a huge step toward better health.
• Avoid Low-Oxygen Environments: If you live at high altitudes, or plan to go skiing or hiking in mountainous areas, be mindful of the lower oxygen levels. Your body may need extra care in these situations.
• Be Mindful of Temperature Extremes: Poor blood flow can make you more vulnerable to injuries from very hot or very cold temperatures. Dress warmly in cold weather, especially on your hands and feet. Protect yourself from the sun and drink plenty of fluids in hot weather.
• Monitor for Sores: Slow healing of sores, especially on your hands and feet, is a potential concern. Regularly check your feet and hands for any sores or wounds and report any problems to your doctor promptly.

By following these tips, you can actively manage your polycythemia vera and improve your overall well-being. Remember to discuss any concerns or changes in your health with your doctor.