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Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder characterized by too few platelets in the blood. This is because platelets are being destroyed by the immune system. Idiopathic means the exact cause of the disease is unknown.
Platelets (thrombocytes) are colorless blood cells that stop blood loss by clumping together at the site of a blood vessel injury and forming plugs in vessel holes.
People with idiopathic thrombocytopenic purpura, because of their low platelet count, tend to bruise easily and bleed longer when injured. Nosebleeds and bleeding gums also are common.
Idiopathic (id-ee-o-PATH-ick) means that the cause of the disease or condition isn’t known. Thrombocytopenic (throm-bo-cy-toe-PEE-nick) means there is a lower-than-normal number of platelets in the blood. Purpura (PURR-purr-ah) are purple bruises caused by bleeding under the skin. More extensive bleeding can create a three-dimensional mass called a hematoma (he-ma-TO-ma).
Types of Idiopathic Thrombocytopenic Purpura:
Idiopathic thrombocytopenic purpura is often divided into two categories: acute and chronic. Acute ITP is the most common form and occurs most frequently in children, typically after a viral infection. It usually goes away on its own within six months. Chronic ITP lasts longer than six months and is more common in adults.Although most cases are asymptomatic, very low platelet counts can lead to a bleeding diathesis and purpura.
Immune thrombocytopenic purpura; ITP,essential thrombocytopenia, haemogenia, haemogenic syndrome, haemorrhagic purpura, idiopathic thrombopenic purpura, morbus haemorrhagicus maculosus, morbus maculosis haemorrhagicus, morbus maculosus werlhofii, peliosis werlhofi, primary splenic thrombocytopenia, primary thrombocytopenia, primary thrombocytopenic purpura, purpura haemorrhagica, purpura thrombocytopenica, purpura werlhofii, splenic thrombocytopenic purpura, thrombocytolytic purpura.
For most children and adults, ITP isn’t a serious or life-threatening condition.
Acute ITP 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 ITP, the platelet count returns to normal within 6 to 12 months. Treatment may not be needed. A small number of children, about 5 percent, whose ITP doesn’t go away on its own may need to have further medical or surgical treatment.
Chronic ITP will vary with each individual and can last many years. Even people who have severe forms of chronic ITP can live for decades. Most people with chronic ITP are able at some point to stop treatment and keep a safe platelet count.
In most cases, it’s believed that the body’s immune system causes idiopathic thrombocytopenic purpura (ITP). Normally, the immune system makes antibodies to fight off germs or other harmful things that enter the body. In ITP, however, the immune system treats your own platelets as if they were invaders in the body, attacking and destroying them.
The reason why the immune system decides to attack its own platelets isn’t known.
Children who get the acute (temporary) type of ITP often have had a recent viral infection. It’s possible that the infection somehow “triggers” or sets off the immune reaction that leads to ITP in these children. ITP in adults, on the other hand, doesn’t seem to be linked to infections.
The disease occurs when immune system cells, called lymphocytes, produce antibodies against platelets. Platelets are necessary for normal blood clotting. They clump together to plug small holes in damaged blood vessels.
The presence of antibodies on platelets leads to their destruction in the spleen. A characteristic skin rash, easy bruising, abnormal menstrual bleeding, or sudden and severe loss of blood from the digestive tract may occur.
Usually, no other abnormal findings are present. In children, the disease sometimes follows a viral infection, and usually runs its course without treatment. In adults, it is more often a chronic (long-term) disease and can follow a viral infection, taking certain drugs, pregnancy, or other immune disorders.
ITP affects women more frequently than men, and is more common in children than adults. in Children, equal numbers of boys and girls are affected.
Antibodies produced by your immune system attach themselves to the platelets, marking the platelets for destruction. 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 platelets than normal.
Normally, you have anywhere from 150,000 to 450,000 platelets per microliter of circulating blood. As the number of your platelets decreases, your risk of bleeding increases. The greatest risk is when platelet count falls very low — below 10,000 platelets per microliter. At this point, internal bleeding may occur despite a lack of any injury, although this is rare.
In children with acute ITP, the disorder often follows a viral illness, such as the mumps, a respiratory infection or a flu-like illness. It may be that such an infection sets off the immune system, triggering it to malfunction. But ITP in adults isn’t linked to infections.
Who Is At Risk:
Both children and adults can develop idiopathic thrombocytopenic purpura (ITP).
Children usually get the acute (temporary) type of ITP. This type of ITP often develops after an infection caused by a virus. Adults tend to get the chronic (long-lasting) type of ITP. Women are 2 to 3 times more likely than men to get chronic ITP.
ITP is a fairly common blood disorder, with 50 to 150 new cases per every 1 million people each year; about half of these cases are in children. However, the number of cases of ITP is increasing because routine blood tests that show a low platelet count are being done more often.
You can’t catch ITP from another person.
Signs & Symptoms:
Having a low platelet count doesn’t cause symptoms. The bleeding caused by a low platelet count may have the following signs or symptoms.
Signs that typically indicate a low platelet count and possibly idiopathic thrombocytopenic purpura include:
* Easy or excessive bruising (purpura)
* Superficial bleeding into your skin that appears as a rash of pinpoint-sized reddish-purple spots (petechiae), usually on your lower legs
* Prolonged bleeding from cuts
* Unusually heavy menstrual flows
* Profuse bleeding during surgery
* Pinpoint red spots on the skin (frequently the legs) that can often be found in groups and may look like a rash. The spots, called petechiae, are due to bleeding under the skin.
* Bruising or purplish areas on the skin or mucous membranes (such as in the mouth) due to bleeding under the skin. The bruises may occur for no apparent reason. This type of bruising is called purpura. More extensive bleeding can create a three-dimensional mass called a hematoma.
* Nosebleeds or bleeding from the gums (for example, when dental work is done).
* Blood in the urine or stool (bowel movement).
* Abnormally heavy menstruation
Bleeding in the brain is rare, and the symptoms of bleeding in the brain may vary in severity.
A low number of platelets won’t cause pain, fatigue (tiredness), difficulty concentrating, or any other symptoms
The incidence of ITP is 50–100 new cases per million per year, with children accounting for half of that amount.
More than 70% of the cases in children end up in remission within 6 months whether treated or not. Moreover, a third of the remaining chronic cases remitted during the follow-up observation, and another third ended up with only mild thrombocytopenia (>50,000 platelets per µL). ITP is usually chronic in adults and the probability of durable remission is 20–40%. The male:female ratio in the adult group is 1:1.2–1.7 (for children it is 1:1) and the median age of adults at the diagnosis is 56–60.
Usually, ITP patients suffer from bruising; petechiae, nosebleeds and bleeding gums may occur if the platelet count is below 20,000, compared to a normal range of 150,000–400,000 per mm3.
Subarachnoid, intracerebral hemorrhage or other internal bleeding are very serious possible complications of this disease. Fortunately, these are unlikely in patients with the platelets count above 20,000.
To diagnose idiopathic thrombocytopenic purpura (ITP), your doctor must first be certain that your low platelet count isn’t caused by other conditions, such as HIV infection or lupus, or by medicines (for example, chemotherapy drugs or aspirin). Your doctor will ask about your medical history, do a physical exam, and order blood tests to measure the platelet count in your blood. These tests usually include:
* A complete blood count. This test shows the numbers of different kinds of blood cells, including platelets, in a small sample of your blood. In ITP, the red and white blood cell counts are normal.
* A blood smear. During this test, some of your blood is put on a slide. A microscope is then used to look at your platelets and other blood cells.
* Some laboratories may be able to test for the antibodies that attack platelets.
The diagnosis of ITP is one of exclusion. First, one has to make sure that there are no other blood abnormalities except for low platelet count and no physical signs except for signs of bleeding. Then, the secondary causes (usually 5-10% of suspected ITP cases) should be excluded. Secondary causes could be leukemia, medications (e.g. quinine, heparin), lupus erythematosus, cirrhosis, HIV, hepatitis C, congenital causes, antiphospholipid syndrome, von Willebrand factor deficiency and others. In approximately 1% of cases autoimmune hemolytic anemia and immune thrombocytic purpura coexist, which is a condition called Evans syndrome.
Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged. In fact, an enlarged spleen should lead a clinician to investigate other possible causes for the thrombocytopenia.
Bleeding time is prolonged in ITP patients; however, the use of bleeding time in diagnosis is discouraged by the American Society of Hematology practice guidelines as useless. For example the BMJ review of the basics of hematology states: “The bleeding time may or may not be prolonged in congenital or acquired platelet dysfunction, and therefore a normal bleeding time does not exclude these conditions.”
A bone marrow examination may be performed on patients over the age of 60 and people who do not respond to treatment, or when the diagnosis is in doubt. On examination of the bone marrow, an increase in the production of megakaryotes is seen and can help in determining if it’s ITP. The blood analysis for the antiplatelet antibodies is a matter of clinician’s preference, as there is a disagreement whether the 80% specificity of this test is sufficient.
A specialist, such as a doctor who specializes in blood disorders (hematologist) or in cancer (oncologist), usually performs this test. A needle is used to draw a sample of bone marrow cells for examination under a microscope.
With ITP, however, your bone marrow is normal because the low platelet count is caused by immune-related destruction of platelets, not by impaired production of platelets.
In children, the disease often runs its course without treatment.
Treatment for idiopathic thrombocytopenic purpura (ITP) is based on how much and how often you’re bleeding and your platelet count. Medicines are often used as the first course of treatment. Treatments used for children and adults are similar.
Adults with ITP who have very low platelet counts or problems with bleeding are usually treated. Adults who have milder cases of ITP may not need any treatment, other than monitoring their symptoms and platelet counts.
The acute (short-term) type of ITP that occurs in children often goes away within a few weeks or months. Children who have bleeding symptoms, other than merely bruising (purpura), are usually treated. Milder cases in children may not need treatment other than monitoring and followup to be sure platelet counts return to normal.
If treatment is needed for adults or children who have ITP, medicines are usually used first. Corticosteroids (cor-ti-co-STEER-roids), such as prednisone, are commonly used to treat ITP. These medicines, called steroids for short, help raise the platelet count in the blood by lowering the activity of the immune system. However, steroids have a number of side effects, and some people relapse (get worse) when treatment ends.
Some medicines used to help raise the platelet count are given through a needle in a vein. These medicines include immune globulin and anti-Rh (D) immunoglobulin.
Experimental medicines may be tried if these treatments don’t help. Medicines may be used along with a procedure to remove the spleen, called splenectomy (splee-NECK-tuh-mee).
Removal of the Spleen (Splenectomy)
If necessary, the spleen will be removed surgically. This organ is located in the upper left abdomen and is about the size of a golf ball in children and a baseball in adults. If ITP hasn’t responded to steroids, removing the spleen will reduce the destruction of platelets, but it also may make you more likely to get certain types of infections. If your spleen is removed, discuss with your doctor steps you can take to help avoid these infections and the symptoms to watch out for.
A relatively new strategy is treatment with anti-D, but the patient must be Rh+. This treatment (with products such as Rhophylac and RhoGAM) is normally administered to Rh- women during pregnancy and after the birth of an Rh+ baby to prevent sensitization to the Rh factor..
Some people with ITP who have severe bleeding may need to have platelet transfusions and be hospitalized. Some will need a platelet transfusion before having surgery. For a platelet transfusion, donor platelets from a blood bank are injected into the recipient’s bloodstream to increase the platelet count for a short time.
Some types of infections can briefly lower a person’s platelet count. If a person with ITP has an infection that can lower the number of platelets, treating the infection may help raise the platelet count and reduce bleeding problems.
* Dapsone (also called Diphenylsulfone, DDS, or Avlosulfon) is an anti-infective sulfone drug. In recent years Dapsone has also proved helpful in treating lupus, rheumatoid arthritis and as a second-line treatment for ITP. The exact mechanism by which Dapsone assists in ITP is unclear. However, limited studies report successful increases in platelet counts of around 40–50% of patients taking the drug.
* Romiplostim (also known as AMG531, trade name Nplate) is an experimental treatment for stimulating platelet production. It is a thrombopoiesis stimulating Fc-peptide fusion protein (peptibody). Initial clinical trials show it to be effective in chronic ITP. . It is currently awaiting FDA-approval.
* The novel agent eltrombopag has been demonstrated to increase platelet counts and decrease bleeding in a dose-dependent manner.
* Promising results have been reported in a small phase II study of an experimental kinase inhibitor tamatinib fosdium (R788). 9 out of 14 patients refractory to other treatments (10 of them failed splenectomy) responded to tamatinib. 6 patients achieved >100,000 platelets/uL count.
H. pylori eradication
Researchers in Japan (including Ryugo Sato, Oita University) and Italy (including Massimo Franchini, University of Verona) have found a possible connection between H. pylori (Helicobacter pylori) infection and ITP. Some patients given antibiotic treatment to eradicate the bacterial infection have had their platelet count increase dramatically
If a person who has ITP is taking medicine that can lower the number of platelets or cause bleeding, stopping the medicine can sometimes help raise the platelet count or prevent bleeding. For example, aspirin and ibuprofen are common medicines that reduce platelet function and increase the chance of bleeding. If you have ITP, you shouldn’t use these medicines.
You can’t prevent idiopathic thrombocytopenic purpura (ITP) from occurring, but you can prevent its complications.
- Avoid medicines such as aspirin or ibuprofen that can affect your platelets and increase your risk for bleeding.
- Protect yourself from injuries that can cause bruising or bleeding.
- Seek treatment right away for any infections you may develop. Report any symptoms of infection, such as a fever, to your doctor. This is especially important for adults or children with ITP who have had their spleens removed.
.Living With Idiopathic Thrombocytopenic Purpura:
If you have idiopathic thrombocytopenic purpura (ITP):
* Stay away from medicines that may affect platelets and increase your chance of bleeding. Common examples are aspirin or ibuprofen. Be careful when using over-the-counter medicines, as many of them do contain aspirin or ibuprofen. Tell your doctor about all of the over-the-counter medicines you take, including vitamins, supplements, or herbal remedies, as they could contain substances that increase your risk of bleeding.
* Avoid injuries that can cause bruising and bleeding. Take precautions such as regular use of seatbelts and wearing gloves when working with knives and other tools. If your child has ITP, protecting him or her from injuries, especially head injuries that could cause bleeding in the brain, is important. Ask your child’s doctor whether you need to restrict your child’s activities.
* Find a doctor who is familiar with treating ITP patients. Hematologists are doctors who specialize in diagnosing and treating blood diseases and disorders.
* Watch for symptoms of infection, such as fever, and report them to your doctor promptly. If you have had your spleen removed, you may be more likely to become ill from certain types of infections. People who have had a splenectomy should receive certain vaccinations to prevent these infections. Your doctor can tell you which vaccinations you should have.
Idiopathic Thrombocytopenic Purpura in Pregnancy:
In women who are pregnant and have ITP, the ITP usually doesn’t affect the baby. However, some babies born to mothers with ITP are born with or develop low numbers of platelets soon after birth. Their platelets almost always return to normal without any treatment. Treatment can speed the recovery in the few babies whose platelet counts are very low.
Treatment for ITP during pregnancy depends on the 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 milder cases of ITP can usually go through pregnancy without treatment. Pregnant women with very low platelet counts or a lot of bleeding are more likely to have serious heavy bleeding during delivery or afterward. To prevent serious bleeding, these women are usually treated.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose