Polycythemia Vera

What Is Polycythemia Vera?

Polycythemia vera (PV) is a blood cancer that begins in the marrow of your bones, the soft center where new blood cells grow. It causes your marrow to make too many red blood cells so your blood is too thick. You may be more likely to have clots, a stroke, or a heart attack.

This disease gets worse slowly, usually over many years. It can be life- threatening if you don't get treatment, but the right care can help you live a long life.

Most people who have PV don’t get diagnosed until they’re 60 or older, usually after a routine blood test. But it can happen at any age. Men get it more often than women.

It's common to worry when you find out that you have cancer. Remember that everyone is different and that all cancers aren’t the same. With the support of your doctor, family, friends, and other people who have polycythemia vera, you’ll be in the best position to manage it.

Polycythemia Vera Causes

Your bone marrow makes three types of blood cells:

Red
• White
• Platelets

Diffuse large B-cell lymphoma,

or DLBCL, is a common type

of fast-growing blood cancer.

It happens when your B cells,

a kind of white blood cell,

grow and multiply out

of control.

These cancerous B cells, called

lymphocytes, can build up

in your lymph nodes

or travel to other parts

of your body and form tumors.

CAR T-cell therapy, a type

of gene therapy,

uses your immune system to fight

the lymphoma.

Genes are made up of DNA,

the code that tells your cells

what to do.

Genes that don't work the way

they should can cause diseases.

First, your doctor collects some

of your T cells,

another kind of white blood

cell,

and changes the genetic code

by putting in a new gene.

When your T cells are put back

inside your body, they are now

programmed to target and kill

cancerous lymphocytes.

Red blood cells carry oxygen, white ones fight infections, and platelets clot your blood to stop bleeding.

Most people with polycythemia vera have too many red blood cells. But it can also cause you to have too many white blood cells and platelets.

PV is caused by a gene (either JAK2 or TET2) that doesn’t work the way it should. Most likely, the problem happened over the course of your life. Rarely, parents can pass these faulty genes to children.

Polycythemia Vera Symptoms

At first, you may not notice any problems. When they do show up, polycythemia vera symptoms can include:

SPEAKER: Sure, the smell

of bacon in the morning

is great and all.

But nothing beats coffee.

But is it good for you?

Well, let's get to the truth

about coffee.

Can you get addicted to coffee?

No.

Now, if you've been hopped up

on caffeine for years,

and one day you suddenly switch

to decaf, your head's

going to hurt.

But the withdrawal is only

temporary.

And most experts don't think

caffeine addiction is

a big problem.

It's not like you're going

to need rehab.

Does decaf mean it has

no caffeine?

Not quite.

A regular cup of coffee

has around 130 milligrams

of caffeine.

A cup of decaf has about five

milligrams.

That's about the same amount

as a cup of hot cocoa or half

of a bar of chocolate.

Should I ever cut back

on coffee?

Sometimes.

If you have osteoporosis,

keep your intake to three cups

a day.

And cut out the caffeine if you

have insomnia.

Also, while caffeine can get rid

of headaches, bigger coffee

drinkers have a bigger chance

of getting migraines.

So if you drink another cup,

that relief might only

be temporary.

And finally, here

is the big one.

Is drinking coffee every day OK?

Yes.

It turns out if you drink up

to three eight-ounce cups

of coffee every day,

you actually get a lot of health

benefits.

And we're not talking

little things.

No.

Coffee drinkers seem more

protected against big things

like diabetes, Alzheimer's

disease, Parkinson's disease,

liver disease, and gout.

The truth is, coffee is good.

So go ahead and fill her up.

• Headaches
• Double vision
• Dark or blind spots in your vision that come and go
• Itching all over your body, especially after you've been in warm or hot water
• Sweating, especially at night
• A red face that looks like sunburn or blushing
• Weakness
• Dizziness
• Weight loss
• Shortness of breath
• Tingling or burning in your hands or feet
• Painful joint swelling

You may also feel pressure or fullness below your ribs on your left side. This might mean PV has enlarged your spleen, an organ that helps filter your blood.

Polycythemia Vera Complications

Blood clots can cause a heart attack, a stroke, or deep vein thrombosis (DVT). They might also make your liver or spleen larger. If your organs don’t get enough blood, you could have chest pain or heart failure.

Having too many red blood cells might cause stomach ulcers, gout, or kidney stones.

PV can also lead to more serious blood diseases like acute leukemia or myelofibrosis. Acute leukemia is a blood cancer that gets worse quickly. Myelofibrosis is a condition in which your bone marrow fills with scar tissue.

Polycythemia Vera Diagnosis

Your doctor will start with a physical exam, including a check of your spleen. They’ll ask about your symptoms and see if your face is unusually red.

You may have some blood tests, including:

• Complete blood count(CBC). Your doctor takes a sample of your blood and sends it to a lab, where a machine counts the number of red blood cells, white blood cells, and platelets. An unusually high number of any of these could be a sign of polycythemia vera.
• Blood smear. Your doctor looks at a sample of your blood through a microscope. It's a way to check for other diseases that are sometimes linked to PV.
• EPO level. This test measures how much of the hormone EPO is in your blood. EPO tells your bone marrow to make blood cells. People who have polycythemia vera have very low amounts of it.

Polycythemia Vera Treatment

If you don't have many symptoms, you might not need treatment for PV right away. Your doctor will keep a close watch on you.

If you do need treatment, it will be aimed at lowering the amount of red blood cells your body makes and preventing blood clots and other complications.

Your options include:

Phlebotomy. This is often the first treatment for people who have polycythemia vera.

Your doctor takes blood from your vein so you have fewer blood cells. It's a lot like donating blood. After it's done, your blood will be thinner, and it’ll flow more easily. You'll usually feel better, too. Some symptoms will ease, like headaches or dizziness.

Your doctor will decide how often you need phlebotomy. Some people with PV don’t need any other treatment for many years.

Low-dose aspirin. This keeps platelets from sticking together. That makes you less likely to get blood clots, which in turn makes heart attacks or strokes less likely. Most people with polycythemia vera take low-dose aspirin.

Lifestyle Changes

Keep yourself comfortable and as healthy as possible during treatment:

• Don't smoke or chew tobacco. Tobacco narrows blood vessels, which can make blood clots more likely.
• Get light exercise, such as walking, to help your circulation and keep your heart healthy.
• Do leg and ankle exercises to keep clots from forming in the veins of your legs. Your doctor or a physical therapist can show you how.
• Bathe or shower in cool water if warm water makes you itch.
• Keep your skin moist with lotion, and try not to scratch.

Polycythemia Vera Prognosis

There's no cure, but the right treatment can help you manage this disease for many years.

Talk to your friends and family about what you’re dealing with. You may also want to join a support group for people who’ve been in your position.

The MPN Research Foundation has more information about polycythemia vera. It can also help you find support groups.

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