Thrombocytopenia and ITP

Thrombocytopenia and ITP

If you have thrombocytopenia, you don’t have enough platelets in your blood. Platelets help your blood clot, which stops bleeding.

For most people, it’s not a big problem. But if you have a severe form, you can bleed spontaneously in your eyes, gums, or bladder or bleed too much when you’re injured.

A healthy person usually has a platelet count of 150,000 to 400,000. You have thrombocytopenia if your number falls under 150,000.

If you're wondering what the long name means, here's how it breaks down: "Thrombocytes" are your platelets, and "penia" means you don't have enough of something. Put those terms together, and you get "thrombocytopenia."

Causes

Thrombocytopenia has many causes. One of the most common causes of low platelets is a condition called immune thrombocytopenia (ITP). You may hear it called by its old name, idiopathic thrombocytopenic purpura. Although doctors don't know what causes primary ITP, they know that it happens when your immune system -- your body's main defense against disease -- doesn't work right. Your antibodies, which are supposed to attack infections, instead mistakenly destroy your platelets.

Thrombocytopenia can run in families, but you can also get it from many medical conditions. Treating the medical condition may improve ITP.

Secondary ITP happens when ITP is linked to another condition, such as:

• Viral infections (including chickenpox, parvovirus, hepatitis C, Epstein- Barr, and HIV)
• Systemic lupus erythematosus (SLE)
• Chronic lymphocytic leukemia (CLL)
• Drug-induced immune thrombocytopenia
• Sepsis, a severe bacterial infection in your blood
• Helicobacter pylori (H. pylori), a bacteria that can live in your digestive system

Medicines Linked to ITP

Some medicines can increase your risk of ITP, such as:

• Certain drugs for heart problems, seizures, and infections
• Heparin, a blood thinner used to prevent blood clots

Work with your doctor to figure out if a drug is causing your platelet count to drop. They may be able to adjust your dose or change your medication.

Other Treatments Linked to ITP

• Heart bypass surgery
• Radiation treatment on your bone marrow

Symptoms

Usually, thrombocytopenia has no symptoms. But when you do have them, they can include:

• Bleeding, most often from the gums or nose. Women with thrombocytopenia may have heavier or longer periods or breakthrough bleeding. You may also see blood in your pee or poop.
• Red, flat spots on your skin, about the size of a pinhead. You see these mostly on your legs and feet, and they may appear in clumps. Your doctor may call them petechiae.
• Blotches and bruises. You might have large areas of bleeding under the skin that don't turn white when you press on them. You also might see what look like the bruises you get from a bump or being hit. They could be blue or purple and change to yellow or green over time. These are caused from the inside, by the sudden leaking from tiny blood vessels. The medical name for these is purpura.

Severe thrombocytopenia can cause a lot of bleeding after an injury, such as a fall.

Getting a Diagnosis

Thrombocytopenia is often found by chance when your doctor does a routine blood test. They might ask you:

• What symptoms (including bleeding) have you noticed?
• When did you first see them?
• Does anything make them better? Or worse?
• What medications and supplements are you taking?
• Have you had any shots in the last month, a blood transfusion, or used drugs with a needle?
• Does anyone in your family have a problem with their immune system, bleeding, or bruising?
• What have you eaten recently?

Your doctor may do a physical exam to check you for signs of bleeding and feel if your spleen seems big.

Some tests check for low platelet levels:

• CBC (complete blood count). This measures the amount of your red and white blood cells and platelets.
• Blood smear. This shows how your platelets look under a microscope.
• Bone marrow test. Your doctor uses a very fine needle to draw a small amount of liquid bone marrow and check it for cells that may not be working right. Or you may get a biopsy, using a different kind of needle, so your doctor can check the types and numbers of cells in the bone marrow.

You may need more tests to help your doctor figure out what's going on.

Treatment

There are a lot of treatment options for thrombocytopenia and ITP. You'll need to work with your doctor to weigh the pros and cons of each and find a therapy that's right for you.

If you have ITP, your treatment depends on how severe a case you have. If it's mild, you may only need to get regular checks of your platelet levels.

When you do need treatment, the goal is to get your platelet count to a level that's high enough to prevent serious bleeding in the gut or brain.

Your doctor will likely suggest these treatments for ITP first:

Corticosteroids.Dexamethasone or prednisone is typically prescribed to raise your platelet count. You take it once a day in the form of a pill or tablet. An increased or normalized platelet count is generally seen within 2 weeks of therapy, particularly with high-dose dexamethasone. Your doctor will then likely cut your dose gradually over the next 4 to 8 weeks. The treatment may have to be repeated, but once your platelet count is normal, none is needed again.

There are some side effects to prednisone, especially if you use it for a long time. Even after a short time, you can get irritable, have stomach upsets, and have other problems such as:

• Sleep problems
• Weight gain
• Puffy cheeks
• Peeing a lot
• Lower bone density
Acne

Besides side effects, another disadvantage to prednisone is that your platelet count may drop once you've finished your treatment.

IVIG (intravenous immune globulin). If you can't get your platelet count up with prednisone, if you cannot tolerate steroids, or if your count drops after you're done with your treatment, your doctor may suggest IVIG. You take this medication through an IV, usually for several hours a day for 1 to 5 days.

The advantage of IVIG is that it can raise your platelet count quickly. But the increase in platelets is only temporary. It is useful for people who need to get their levels boosted fast or who cannot tolerate steroids. The side effects include:

• Nausea and vomiting
• Headaches
• Fever and chills

Surgery. If you have ITP and other treatments haven't raised your platelet levels enough, you may benefit from an operation to remove your spleen. That's the organ that destroys your platelets, so taking it out can give your platelet count a boost. But this doesn't always work.

Getting your spleen removed can make it harder for you to fight infections. Your infection risk is greatest in the first 3 months after your surgery.

Rituximab(Rituxan). This drug is a type of treatment known as biologic therapy. It attacks B cells, a type of white blood cell that can destroy platelets. It's sometimes used if you have severe ITP despite treatment with steroids and you aren't able to have surgery to remove your spleen. Your doctor may also suggest it if you've had your spleen removed but you still have low platelet counts.

Side effects for rituximab include:

• Fever
• Chills
• Weakness
• Nausea
• Headaches
• Weakened immune system

Rho(D) immune globulin. This treatment, which you also take through an IV, is an alternative to traditional IVIG in people who have Rh+ blood. It generally takes less than half an hour. The side effects are similar to IVIG.

If corticosteroids, IVIG, and Rho(D) aren't improving your platelet count and you're having bleeding problems, your doctor may switch to a second set of options. There are pros and cons for each. They include:

Thrombopoietin (TPO) receptor agonists. These drugs are also called platelet growth factors. If you have severely low platelets even after treatment with steroids, surgery to remove the spleen, or rituximab, you will likely do well on these medicines, but you may need to take them long-term.

A TPO drug may also be used in someone who needs a boost in platelet count for a period of time, such as during an acute bleeding episode, in preparation for elective surgery, or while deciding about, planning, or awaiting a splenectomy.

Three TPO drugs are available and include: avatrombopag (Doptelet)., eltrombopag (Promacta), and romiplostim (Nplate).

Eltrombopag is a once-daily pill, romiplostim is taken by shot once a week, and avatrombopag (Doptelet) is taken once a day and then the dose is adjusted to your platelet count.

They get your bone marrow to make more platelets. Side effects include nausea, vomiting, headache, and a higher chance of getting blood clots.

If you’ve tried those and still can't get your platelet count to the right level, your doctor may suggest these medicines:

A newer drug called fostamatinib (Tavalisse), a spleen tyrosine kinase inhibitor, is designed to treat thrombocytopenia in adults with chronic ITP who haven't gotten better with other treatments. The initial dose is a pill twice a day.

Immunosuppressants, such as azathioprine (Imuran), cyclosporine, and mycophenolate mofetil (CellCept). They work by keeping your immune system in check.

Androgens, such as danazol (Danocrine). It’s not used in women because it can cause unwanted hair growth called hirsutism.

Vinca alkaloids, such as vinblastine, vincristine (Vincasar), and, rarely, cyclophosphamide (Cytoxan). Doctors sometimes suggest these if you're having severe bleeding and your platelet count isn't getting a boost from other treatments.

Taking Care of Yourself

You can still do a lot of things, but you may need to make some changes to your lifestyle to prevent getting hurt or cut. For instance, avoid sports such as football and downhill skiing.

Eat a healthy diet with lots of fruits and vegetables, especially leafy greens, to give your body the nutrients it needs. Ask your doctor if you should avoid food with quinine and aspartame, like tonic water, bitter lemon, bitter melon, some diet sodas, and sugar-free foods.

You probably shouldn't take medicines that make bleeding easier, such as aspirin and ibuprofen.

Check with your doctor to see if it's OK for you to drink alcohol, and if it is, how much.

What to Expect

Your case may be different from someone else's. Your doctor will watch you to see how you're doing. If your case is mild, you may not need any treatment. But even people who do need treatment can lead full lives.

Find out as much as you can about your condition so you can best manage it.

Getting Support

The Platelet Disorder Support Association has information about ITP and ways to connect with others who also have it.

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