What is idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder due to unusually low levels of platelets that can lead to easy or excessive bruising and bleeding. Idiopathic thrombocytopenic purpura, which is also called immune thrombocytopenia or autoimmune thrombocytopenic purpura, in which the immune system destroys platelets, which are necessary for normal blood clotting. Platelets are made in your bone marrow along with other kinds of blood cells. Platelets stick together (clot) to seal small cuts or breaks on blood vessel walls and stop bleeding. Without enough platelets, bleeding can occur inside the body (internal bleeding) or underneath or from the skin (external bleeding).

Idiopathic thrombocytopenic purpura (ITP) affects children and adults. Children often develop idiopathic thrombocytopenic purpura after a viral infection and usually recover fully without treatment, also called acute idiopathic thrombocytopenic purpura. In adults, idiopathic thrombocytopenic purpura is often long term or chronic idiopathic thrombocytopenic purpura.

In most cases, an autoimmune response is thought to cause idiopathic thrombocytopenic purpura. Normally, your immune system helps your body fight off infections and diseases. But if you have idiopathic thrombocytopenic purpura , your immune system attacks and destroys its own platelets. The reason why this happens isn’t known.

The spleen, which helps your body fight infection, recognizes the antibodies and removes the platelets from your system. The result of this case of mistaken identity is a lower number of circulating platelets than is normal.

A normal platelet count is generally between 150,000 and 450,000 platelets per microliter of circulating blood. People with ITP often have platelet counts below 20,000. Because platelets help the blood clot, as their number decreases, your risk of bleeding increases. The greatest risk is when your platelet count falls very low — below 10,000 platelets per microliter. At this point, internal bleeding may occur even without any injury.

Idiopathic thrombocytopenic purpura can’t be passed from one person to another.

Any kind of bleeding that’s hard to stop could be a sign of idiopathic thrombocytopenic purpura. This includes menstrual bleeding that’s heavier than normal. Bleeding in the brain is rare, and its symptoms may vary.

Idiopathic thrombocytopenic purpura symptoms may include bruising, nosebleed or bleeding in the mouth, bleeding into the skin, and abnormally heavy menstruation.

Blood tests will be done to check your platelet count. A bone marrow aspiration or biopsy may also be done.

If you don’t have signs of bleeding and your platelet count isn’t too low, you may not need any treatment. In rare cases, the number of platelets may be so low that dangerous internal bleeding occurs. Treatment options are available. With treatment, the chance of remission (a symptom-free period) is good. Rarely, idiopathic thrombocytopenic purpura may become a chronic ailment in adults and reappear, even after remission ¹.

One in 10,000 children have idiopathic thrombocytopenic purpura, so it is quite rare. In children, ITP usually goes away without treatment. Some children may need treatment.

Adults are usually started on a steroid medicine called prednisone. In some cases, surgery to remove the spleen (splenectomy) is recommended. This increases the platelet count in about half of people. However, other drug treatments are usually recommended instead.

If the disease does not get better with prednisone, other treatments may include:

• Medicine called danazol (Danocrine) taken by mouth
• Infusions of high-dose gamma globulin (an immune factor)
• Drugs that suppress the immune system
• Anti-RhD therapy for people with certain blood types
• Drugs that stimulate the bone marrow to make more platelets

People with idiopathic thrombocytopenic purpura should not take aspirin, ibuprofen, or warfarin, because these drugs interfere with platelet function or blood clotting, and bleeding may occur.

Is idiopathic thrombocytopenic purpura hereditary?

The cause of idiopathic thrombocytopenic purpura (ITP) is unknown. It is not thought to be genetic because it is rare for multiple people in the same family to develop this disease ². Only a few cases of familial ITP have been documented, including an affected woman and 3 of her 4 children, identical twins with chronic ITP, and a mother with chronic ITP who had a child with purpura. The child’s purpura resolved on its own within 3 weeks while the mother remained thrombocytopenic ³.

Idiopathic thrombocytopenic purpura types

The two types of idiopathic thrombocytopenic purpura are acute (temporary or short-term) and chronic (long-lasting).

Acute idiopathic thrombocytopenic purpura generally lasts less than 6 months. It mainly occurs in children—both boys and girls—and is the most common type of idiopathic thrombocytopenic purpura . Acute idiopathic thrombocytopenic purpura often occurs after a viral infection.

Chronic idiopathic thrombocytopenic purpura lasts 6 months or longer and mostly affects adults. However, some teenagers and children do get this type of idiopathic thrombocytopenic purpura. Chronic idiopathic thrombocytopenic purpura affects women two to three times more often than men.

Treatment depends on the severity of bleeding and the platelet count. In mild cases, treatment may not be needed.

Idiopathic thrombocytopenic purpura in Pregnancy

In women who are pregnant and have idiopathic thrombocytopenic purpura, the idiopathic thrombocytopenic purpura usually doesn’t affect the baby. However, some babies may be born with or develop low platelet counts soon after birth.

The babies’ platelet counts almost always return to normal without any treatment. Treatment can speed up recovery in the babies whose platelet counts are very low.

Treatment for idiopathic thrombocytopenic purpura during pregnancy depends on a woman’s platelet count. If treatment is needed, the doctor will take a close look at the possible effects of the treatment on the unborn baby.

Women who have mild cases of idiopathic thrombocytopenic purpura usually can go through pregnancy without treatment. Pregnant women who have very low platelet counts or a lot of bleeding are more likely to have heavy bleeding during delivery or afterward. To prevent heavy bleeding, these women usually are treated.

Idiopathic thrombocytopenic purpura causes

In most cases, an autoimmune response is thought to cause idiopathic thrombocytopenic purpura (ITP). Normally, your immune system helps your body fight off infections and diseases. In idiopathic thrombocytopenic purpura, however, your immune system attacks and destroys your body’s platelets by mistake. Why this happens isn’t known. Platelets help your blood clot by clumping together to plug small holes in damaged blood vessels. The antibodies attach to the platelets. The body destroys the platelets that carry the antibodies.

Children who have acute (short-term) idiopathic thrombocytopenic purpura often have had recent viral infections such as the mumps or the flu. These infections may “trigger” or set off the immune reaction that leads to idiopathic thrombocytopenic purpura.

In adults, it is more often a long-term (chronic) disease and can occur after a viral infection or bacterial infections, such as HIV, hepatitis C, or H. pylori. Idiopathic thrombocytopenic purpura can also occur following the use of certain drugs, during pregnancy, or as part of an immune disorder.

Idiopathic thrombocytopenic purpura affects women more often than men. It is more common in children than adults. In children, the disease affects boys and girls equally.

Risk factors for idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura is a fairly common blood disorder. Both children and adults can develop idiopathic thrombocytopenic purpura .

Children usually have the acute (short-term) type of idiopathic thrombocytopenic purpura . Acute idiopathic thrombocytopenic purpura often develops after a viral infection.

Adults tend to have the chronic (long-lasting) type of idiopathic thrombocytopenic purpura. Women are two to three times more likely than men to develop chronic idiopathic thrombocytopenic purpura .

The number of cases of idiopathic thrombocytopenic purpura is rising because routine blood tests that can detect a low platelet count are being done more often.

Idiopathic thrombocytopenic purpura can’t be passed from one person to another.

Idiopathic thrombocytopenic purpura symptoms

Idiopathic thrombocytopenic purpura may not cause any signs or symptoms. However, idiopathic thrombocytopenic purpura can cause bleeding inside the body (internal bleeding) or underneath or from the skin (external bleeding). Signs of bleeding may include:

• Abnormally heavy periods in women
• Bleeding into the skin, often around the shins, causing a skin rash that looks like pinpoint purple spots (petechial rash)
• Easy or excessive bruising (purpura)
• Nosebleed or bleeding in the mouth
• Blood in urine or stools

People who have idiopathic thrombocytopenic purpura often have purple bruises called purpura. These bruises appear on the skin or mucous membranes (for example, in the mouth). Bleeding from small blood vessels under the skin causes purpura.

People who have idiopathic thrombocytopenic purpura also may have bleeding that causes tiny red or purple dots on the skin. These pinpoint-sized dots are called petechiae. Petechiae may look like a rash.

People who have idiopathic thrombocytopenic purpura also may have nosebleeds, bleeding from the gums during dental work, or other bleeding that’s hard to stop. Women who have idiopathic thrombocytopenic purpura may have menstrual bleeding that’s heavier than normal.

A lot of bleeding can cause hematomas. A hematoma is a collection of clotted or partially clotted blood under the skin. It looks or feels like a lump.

Bleeding in the brain as a result of idiopathic thrombocytopenic purpura is very rare, but can be life threatening if it occurs.

A low platelet count doesn’t directly cause pain, problems concentrating, or other symptoms. However, a low platelet count might be associated with fatigue (tiredness).

Idiopathic thrombocytopenic purpura possible complications

Sudden and severe loss of blood from the digestive tract may occur. Bleeding into the brain may also occur.

Pregnancy

In pregnant women with idiopathic thrombocytopenic purpura, the condition doesn’t usually affect the baby. But the baby’s platelet count should be tested soon after birth.

If you’re pregnant and your platelet count is very low or you have bleeding, you have a greater risk of heavy bleeding during delivery. In such cases, you and your doctor may discuss treatment to maintain a stable platelet count, taking into account the effects on your baby.

Idiopathic thrombocytopenic purpura diagnosis

Your doctor will diagnose idiopathic thrombocytopenic purpura based on your medical history, a physical exam, and test results.

Your doctor will want to make sure that your low platelet count isn’t due to another condition (such as an infection) or medicines you’re taking (such as chemotherapy medicines or aspirin).

Medical History

Your doctor may ask about:

• Your signs and symptoms of bleeding and any other signs or symptoms you’re having
• Whether you have illnesses that could lower your platelet count or cause bleeding
• Medicines or any over-the-counter supplements or remedies you take that could cause bleeding or lower your platelet count

Physical Exam

During a physical exam, your doctor will look for signs of bleeding and infection. For example, your doctor may look for purplish areas on the skin or mucous membranes and pinpoint red spots on the skin. These are signs of bleeding under the skin.

Diagnostic Tests

You’ll likely have blood tests to check your platelet count. These tests usually include:

• A complete blood count (CBC). This test checks the number of red blood cells, white blood cells, and platelets in your blood. In idiopathic thrombocytopenic purpura , the red and white blood cell counts are normal, but the platelet count is low.
• A blood smear. For this test, some of your blood is put on a slide. A microscope is used to look at your platelets and other blood cells.
• Bone marrow exam. This test may be used to help identify the cause of a low platelet count, though the American Society of Hematology doesn’t recommend this test for children with idiopathic thrombocytopenic purpura. Platelets are produced in the bone marrow — soft, spongy tissue in the center of large bones. In some cases, a sample of bone tissue and the enclosed marrow is removed in a procedure called a bone marrow biopsy. Or your doctor may do a bone marrow aspiration, which removes some of the liquid portion of the marrow. In many cases, both procedures are performed at the same time (bone marrow exam). In people with idiopathic thrombocytopenic purpura, the bone marrow will be normal because a low platelet count is caused by the destruction of platelets in the bloodstream and spleen — not by a problem with the bone marrow.
• You also may have a blood test to check for the antibodies (proteins) that attack platelets.

If blood tests show that your platelet count is low, your doctor may recommend more tests to confirm a diagnosis of idiopathic thrombocytopenic purpura . For example, bone marrow tests can show whether your bone marrow is making enough platelets.

If you’re at risk for HIV, hepatitis C, or H. pylori, your doctor may screen you for these infections, which might be linked to idiopathic thrombocytopenic purpura.

Some people who have mild idiopathic thrombocytopenic purpura have few or no signs of bleeding. They may be diagnosed only if a blood test done for another reason shows that they have low platelet counts.

Idiopathic thrombocytopenic purpura treatment

Treatment for idiopathic thrombocytopenic purpura is based on how much and how often you’re bleeding and your platelet count.

Adults who have mild idiopathic thrombocytopenic purpura may not need any treatment, other than watching their symptoms and platelet counts. Adults who have idiopathic thrombocytopenic purpura with very low platelet counts or bleeding problems often are treated.

The acute (short-term) type of idiopathic thrombocytopenic purpura that occurs in children often goes away within a few weeks or months. Children who have bleeding symptoms, other than merely bruising (purpura), usually are treated.

Children who have mild idiopathic thrombocytopenic purpura may not need treatment other than monitoring and followup to make sure their platelet counts return to normal.

Medicines

Medicines often are used as the first course of treatment for both children and adults.

• Drugs that suppress your immune system. Your doctor will likely start you on an oral corticosteroid, such as prednisone. This drug may help raise your platelet count by decreasing the activity of your immune system. Once your platelet count is back to a safe level, you can gradually discontinue taking the drug under the direction of your doctor. In general, this takes about two to six weeks. The problem is that many adults experience a relapse after discontinuing corticosteroids. A new course of corticosteroids may be pursued, but long-term use of these medications isn’t recommended because of the risk of serious side effects. These include cataracts, high blood sugar, increased risk of infections and thinning of bones (osteoporosis).
• Injections to increase your blood count. If corticosteroids don’t help, your doctor may give you an injection of immune globulin (IVIG) and anti-Rh (D) immunoglobulin. This drug may also be used if you have critical bleeding or need to quickly increase your blood count before surgery. The effect usually wears off in a couple of weeks. Possible side effects include headache, vomiting and low blood pressure.
• Drugs that boost platelet production. Thrombopoietin receptor agonists — such as romiplostim (Nplate) and eltrombopag (Promacta) — help your bone marrow produce more platelets. Possible side effects include headache, dizziness, nausea or vomiting, and an increased risk of blood clots.
• Other immune-suppressing drugs. Rituximab (Rituxan) helps reduce the immune system response that’s damaging platelets, thus raising the platelet count. Possible side effects include low blood pressure, fever, sore throat and rash.

Medicines also may be used with a procedure to remove the spleen called splenectomy.

If medicines or splenectomy don’t help, two newer medicines—eltrombopag and romiplostim—can be used to treat idiopathic thrombocytopenic purpura .

Emergency treatment

Although rare, severe bleeding can occur with idiopathic thrombocytopenic purpura. Emergency care usually includes transfusions of platelet concentrates, intravenous corticosteroid (methylprednisolone) and intravenous immune globulin.

For a platelet transfusion, donor platelets from a blood bank are injected into the recipient’s bloodstream. This increases the platelet count for a short time.

Treatments for resistant disease

If your condition persists despite treatment, your doctor may suggest other drugs that suppress the immune system or boost platelet production:

• Removal of your spleen (splenectomy). If your condition is severe or persists despite initial drug treatment, your doctor may suggest surgical removal of your spleen (splenectomy). The spleen is located in the upper left abdomen. The spleen is about the size of a golf ball in children and a baseball in adults. The spleen makes antibodies (proteins) that help fight infections. In idiopathic thrombocytopenic purpura, these antibodies destroy platelets by mistake. If idiopathic thrombocytopenic purpura hasn’t responded to medicines, removing the spleen will reduce the destruction of platelets. However, it also may raise your risk for infections. Before you have the surgery, your doctor may give you vaccines to help prevent infections. Splenectomy quickly eliminates the main source of platelet destruction in your body and improves your platelet count, though it doesn’t work for everyone. Serious post-surgical complications sometimes occur, and not having a spleen permanently increases your susceptibility to infection. Splenectomy is rarely a treatment choice for children with idiopathic thrombocytopenic purpura because they often get better without treatment.
• Other drugs. Azathioprine (Imuran, Azasan) has been used to treat idiopathic thrombocytopenic purpura. But it can cause significant side effects, and its effectiveness has yet to be proved. Possible side effects include fever, headache, nausea and vomiting, and muscle pain.

Treating Infections

Some infections can briefly lower your platelet count. Treating the infection may help increase your platelet count and reduce bleeding problems.

Stopping Medicines

Some medicines can lower your platelet count or cause bleeding. Stopping the medicine can sometimes help raise your platelet count or prevent bleeding.

For example, aspirin, ibuprofen, ginkgo biloba and warfarin (Coumadin) are common medicines that increase the risk of bleeding. If you have idiopathic thrombocytopenic purpura, your doctor may suggest that you avoid these medicines.

Idiopathic thrombocytopenic purpura prognosis

For most children and adults, idiopathic thrombocytopenic purpura isn’t a serious or life-threatening condition.

Acute idiopathic thrombocytopenic purpura in children often goes away on its own within a few weeks or months and doesn’t return. In 80 percent of children who have idiopathic thrombocytopenic purpura , the platelet count returns to normal within 6 to 12 months. Treatment may not be needed.

For a small number of children, idiopathic thrombocytopenic purpura doesn’t go away on its own and may require further medical or surgical treatment.

Chronic idiopathic thrombocytopenic purpura varies from person to person and can last for many years. Even people who have severe forms of chronic idiopathic thrombocytopenic purpura can live for decades. Most people who have chronic idiopathic thrombocytopenic purpura can stop treatment at some point and maintain a safe platelet count.

Living with idiopathic thrombocytopenic purpura

If you have immune thrombocytopenia (idiopathic thrombocytopenic purpura), you can take steps to prevent complications. Lifestyle changes and ongoing care can help you manage the condition.

Lifestyle changes

Try to avoid injuries, especially head injuries, that can cause bleeding in the brain. For example, don’t take part in contact sports, such as boxing, football, or karate. Other sports, such as skiing or horseback riding, also put you at risk for injuries that can cause bleeding.

Some safe activities are swimming, biking (with a helmet), and walking. Ask your doctor about physical activities that are safe for you.

Take precautions such as regular use of seatbelts and wearing gloves while working with knives and other tools.

If your child has idiopathic thrombocytopenic purpura, ask his or her doctor whether you need to restrict your child’s activities.

Ongoing care

You may want to find a doctor who is familiar with treating people who have idiopathic thrombocytopenic purpura. For example, hematologists are doctors who specialize in diagnosing and treating blood disorders. Discuss with your doctor how to manage idiopathic thrombocytopenic purpura and when to seek medical care.

Talk with your doctor before taking prescription medicines or over-the-counter medicines, supplements, vitamins, or herbal remedies. Some medicines and supplements can affect platelets and increase your chance of bleeding. Common examples are aspirin or ibuprofen. Your doctor may advise you to avoid these medicines.

Watch for symptoms of infection, such as a fever, and report them to your doctor promptly. If you’ve had your spleen removed, you may be more likely to become ill from certain infections.

References

1. Immune thrombocytopenic purpura (ITP). https://medlineplus.gov/ency/article/000535.htm
2. Immune thrombocytopenic purpura. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3002
3. Paul J. Converse et al. THROMBOCYTOPENIC PURPURA, AUTOIMMUNE; AITP. OMIM. March 20, 2009; http://www.ncbi.nlm.nih.gov/omim/188030.