Thrombocytopenia and ITP

What Are 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 too much when you're injured or spontaneously in your eyes, gums, or bladder.

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

There are many causes of thrombocytopenia. Your doctor may tell you that you have a form of the condition called immune thrombocytopenia (ITP), which is one of the most common causes of low platelets in people who do not have symptoms. You may hear it referred to by its old name, idiopathic thrombocytopenic purpura. Although doctors don't know what causes 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. 

Your body might have fewer platelets because of these causes:

Infections

  • Viral infections, including chickenpox, parvovirus, hepatitis C, Epstein-Barr, and HIV
  • Sepsis, a severe bacterial infection in your blood
  • Helicobacter pylori (H. pylori), a bacteria that can live in your digestive system

Medicines

  • Medication side effects, including drugs for heart problems, seizures, and infections
  • Heparin, a blood thinner used to prevent blood clots
  • Chemotherapy

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

Other Treatments

Medical Conditions

  • Blood cancer such as leukemia or lymphoma
  • A problem with your bone marrow, like toxicity from alcohol use
  • Vitamin B12 or folate (vitamin B9) deficiency
  • Pregnancy. Up to 5% of healthy women get it during pregnancy, and it usually gets better on its own after your baby is born. But it can also be a sign of something more concerning, like preeclampsia or HELLP syndrome.
  • An enlarged spleen
  • Your body uses too many platelets, leaving you without enough of them. That can happen if you have an autoimmune disease, like rheumatoid arthritis or lupus.
  • Rare disorders like hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (TTP), [LB5] [AC6] which uses a lot of platelets to make small blood clots throughout your body.

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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. He or she 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 number 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.

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Questions for Your Doctor

  • What's causing the problem?
  • What are my treatment options? Which do you recommend?
  • Do these treatments have side effects? What can I do about them?
  • How will we know if the treatment is working?
  • When will I start to feel better?
  • What do you expect for my case?
  • Does this condition put me at risk for anything else?
  • Do I need to see a specialist?

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 first:

Corticosteroids.  Prednisone is the most common one used to treat ITP. Doctors prescribe it to try to raise your platelet count. You typically take it once a day in the form of a pill or tablet.

Usually you take prednisone until your platelet level improves, typically within 1 or 2 weeks. Your doctor will then likely gradually reduce your dose over the next 4 to 8 weeks.

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
  • Need to pee often
  • Lower bone density
  • Acne

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

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.

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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:

IVIG (intravenous immune globulin). If you can't get your platelet count up [LB9] [AC10] 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 over a period of 1 to 5 days.

The advantage of IVIG is that it can raise your platelet count quickly. The increase in platelets, however, 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:

Rho(D) immune globulin. This treatment, which you also take through an IV, is an alternative to traditional IVIG in patients 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.

Two TPO drugs are available: eltrombopag (Promacta) and romiplostim (Nplate). Eltrombopag is a once-daily pill, and romiplostim is taken by injection once a week. They get your bone marrow to make more platelets. Side effects include nausea, vomiting, and headache, and a higher risk of blood clots.

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

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

Ask your doctor 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.

WebMD Medical Reference Reviewed by Lisa Bernstein, MD on May 04, 2016

Sources

SOURCES:

Hoffman, R. Hematology: Basic Principles and Practice, 5th edition, Churchill Livingstone Elsevier, 2009.

National Institutes of Health: "How Is Thromobocytopenia Diagnosed?" "Living With Thrombocytopenia."

Cleveland Clinic: "Thrombocytopenia."

Platelet Disorder Support Association: "Food as a Cure."

American Academy of Family Physicians: "Idiopathic Thrombocytopenic Purpura (ITP)."

ITP Foundation: "What Is ITP?"

UptoDate: "Immune thrombocytopenia (ITP) in adults."

FDA.

Medscape: "Rituximab: An anti-CD20 Antibody for the Treatment of Chronic Refractory Immune Thrombocytopenic Purpura."

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