What Is ITP?

ITP, or immune thrombocyticpurpura (THROM-boe-see-tic purr-PURR-uh), is a disease that destroys platelets in the blood. Platelets are the blood cells that help form clots to stop bleeding and close up wounds. When the platelet count is low, bleeding too much can be a problem. ITP can also stand for idiopathic thrombocytopenic purpura. Idiopathic means that we do not know what causes it. Thrombocytopenic means that there are not enough platelets in the blood. Purpura means bruises in the skin. The immune system usually fights germs or infections. If your child has ITP, their immune system instead attacks the body s platelets and destroys them. The platelets are mostly destroyed in the spleen.

ITP in Children Any child can get ITP. Even though we are not sure what causes it, ITP sometimes happens when children get some kinds of viruses or take some kinds of medicines. It can be more likely in children with certain kinds of immune disorders. Even though ITP can start with a virus, it is not an infection. Your child cannot not catch ITP from someone else. For most children with ITP, it goes away within six months. Platelet count usually goes back to normal in four to six weeks. When ITP lasts longer than 12 months, it is called chronic ITP. Chronic ITP happens in about one in four children with ITP. Symptoms of ITP Having ITP means your child has fewer platelets than normal. Most symptoms of ITP are from bleeding problems:

Bleeding more easily than usual Bruising or purplish areas on the skin (purpura). These are caused by bleeding under the skin. Tiny round red spots on the skin (petechiae, or puh-TEE-key-eye) that may look like a rash. These are also caused by bleeding under the skin. Nosebleeds Bleeding from the gums and blood blisters in the mouth Blood in your child s pee (urine) or bowel movements (stool) Any bleeding that is difficult to stop Heavy menstrual bleeding

ITP Diagnosis Because there are other health problems that can cause low platelet levels, your child s healthcare team will talk to you in detail about your child s health. They will need to know more than your child s platelet level before they know whether your child has ITP. Your child s provider will ask about your child s symptoms, recent illnesses and medicines that may affect platelets. They will also ask about any immune problems that your child might have.

Your child s healthcare provider will also do the following to find out if your child has ITP:

Look for signs of bleeding, bruising or petechia, or other health problems Do blood tests Sometimes, they may need to do a bone marrow test.

Bone marrow tests If your child needs a bone marrow test, they will get medicine to make them sleep so that they do not feel pain during the procedure. Then, a member of the healthcare team will place a needle into the hip bone and use a syringe to take out a small amount of bone marrow (aspiration). Sometimes, they may also need to take out a very small piece of bone (biopsy). There may be a little soreness in the hip for one to two days after the procedure. ITP Treatment Options Many children with ITP do not need treatment. It often goes away on its own in a few weeks or months.

For more serious cases, your child s provider may recommend treatment. This will not cure ITP, but it will help your child make more platelets and slow down the speed that platelets get destroyed. Here are the main ITP treatment options:

Intravenous gamma globulin (IVIG)

IVIG is made from blood plasma. It may help block the antibodies that cause platelet destruction. It is given into the vein over several hours. IVIG can cause nausea, headache, and other side effects.

WinRho SDF This is also made from blood plasma. It causes the spleen to destroy some of your child s red blood cells instead of platelets. This medicine may cause a temporary drop in red blood cells (anemia), but may help to raise the platelet levels. It is given into the vein over about 15 minutes. Your child s healthcare team will watch your child for several hours after treatment.

Corticosteriods These medicines may help slow down the speed that the spleen takes platelets out of the blood. They are usually taken by mouth. Steroids, like prednisone or dexamethasone, have several side effects. They make your child gain weight, feel hungrier than usual, have an upset stomach or feel moody. If your child gets this treatment, their provider will talk with you about how to manage side effects.

Taking out the spleen (splenectomy) For some very serious or long-lasting cases of ITP, healthcare providers might recommend a splenectomy. This surgery has long-term effects, so it is important to talk with your child s medical provider about what to consider and what to expect.

Other treatments Other medicines are sometimes used for ITP, especially when it is very serious or chronic. Your child s provider may talk with you about these if your child may need them.

Many people wonder about using transfusions as a treatment. We almost never use platelet transfusions in ITP treatment because the transfused platelets are destroyed by the spleen as quickly as the child's own platelets.

Your child s provider will talk with you about when and how often your child s platelet count should be checked. Right after treatment for ITP, we will check your child s platelet count often (every two to

"Idiopathic" means that the cause of the condition is unknown." In people who have ITP. that is usually a time to check the platelet count. But if your child develops new symptoms or has problems like bruising again. This plan will depend on your child s individual needs. or they might have bleeding in the intestines. We will work with you and with your primary care provider to decide where to have those blood tests done and whom to talk to about the results.three days). "Purpura" means a person has excessive bruising. especially on the lower legs Cuts or minor wounds that take a very long time to clot or stop bleeding Blood in the urine or stools . a person who has ITP might have nosebleeds that are hard to stop. Idiopathic Thrombocytopenic Purpura (ITP) What is ITP? ITP stands for idiopathic thrombocytopenic purpura. ITP is also sometimes called "immune thrombocytopenic purpura. When the platelet count is very low. When the platelet count is normal and stable. they may not need to be checked so often. we will check less often. or even bleeding in the brain with minor trauma. A person who has too few platelets bruises very easily and can bleed for a long time after being injured. all of the blood cells are normal except for the platelets. or if they have ITP longer than four to six weeks. Your child s provider will talk with you about the plan to monitor your child. we may check weekly for the next month. Return to top What are the symptoms of ITP? The symptoms of ITP include: Easy or excessive bruising Petechiae (tiny reddish purple dots on the skin that are caused by bleeding under the surface of the skin) on the body. If there are no problems. "Thrombocytopenic" means the blood doesn't have enough platelets (platelets are also called thrombocytes). Platelets are the tiny cells that form blood clots and seal minor cuts and wounds. we can stop checking platelet levels. If your child does not need treatment. After that.

Return to top How does ITP affect children? ITP in children is usually mild and runs it course without the need for treatment. About 80% of children recover completely from ITP within about 6 months.Unusually heavy menstrual flow in women Unexplained bleeding from the nose or gums What causes ITP? The cause of ITP is not known. the antibodies attack the body's own blood platelets. In children. Normally. ITP does not seem to be hereditary (run in families). but it can occur in anyone. One type affects children. Return to top How is ITP treated in children? . Most adults with ITP are young women. antibodies are a healthy response to bacteria or viruses. Return to top How is ITP diagnosed? Your doctor can begin to diagnose ITP by asking questions about your health and doing a physical exam. People who have ITP form antibodies that destroy their blood platelets. Your doctor may also want you to get a bone marrow exam to rule out other possible causes of your symptoms. Return to top Who gets ITP? There are 2 types of ITP. ITP is not contagious (you can t catch it from someone else). and the other type affects adults. the usual age for getting ITP is 2 to 4 years of age. however. Your doctor may take a blood sample for a test that counts blood cells and platelets (called a CBC) or look at it under a microscope (called a blood smear). In people who have ITP.

further reducing surgical risk. In an otherwise healthy person. Prednisone raises the level of your platelet count. Children don't have to go to the hospital if good care is available at home. when the medicine is stopped. In women. As your count rises and reaches a safe level. quite a few people have no bleeding symptoms. They are only diagnosed with ITP when their blood is checked for another reason and a low blood platelet count is found. Return to top How is ITP treated in adults? Treatment of ITP in adults is aimed at increasing the blood platelet count. Some patients might take prednisone for several weeks or months. Your doctor may recommend that you get high doses of immune globulin (through a needle). The spleen makes most of the antibodies that destroy the blood platelets. your platelet count may decrease again. Removing the spleen will permanently reduce your body s ability to fight infection. removal of the spleen is not a serious operation.Because most children recover with no treatment. . this treatment is done less often than it once was. Some people can also relapse (have the condition return) even after the spleen is removed. At the time of diagnosis. most adults have noticed increased bleeding and easy bruising for several weeks or even months. increased menstrual blood flow is a common sign. Many adults have only mild thrombocytopenia. However. In fact. However. Return to top How does ITP affect adults? In most adults. Mild cases of ITP may not require treatment. just regular monitoring of the platelet levels. your doctor may recommend that your spleen be removed. If prednisone doesn't help enough. It also destroys old or damaged blood cells. ITP lasts much longer than it does in children. your doctor may gradually decrease your medicine until you no longer take the prednisone. However. Both medicines have some side effects. many doctors recommend just watching them carefully and taking care of the bleeding symptoms. This is not the same as curing the disease. Laparoscopic removal of the spleen is now possible. some doctors recommend a short treatment with prednisone pills or intravenous infusions (given in a vein) of gamma globulin to increase the platelet count more quickly.

Talk to your doctor if you are taking medicine and want to become pregnant. Diagnosing ITP during pregnancy can be difficult. Return to top What about ITP in pregnant women? Many of the medicines used to treat ITP should not be taken by women who are pregnant.Other treatments for ITP include another kind of steroid medicine called danazol or other medicines that work to hold the immune system in check. such as warfarin (a medicine often used to treat an abnormal heart rhythm called atrial fibrillation) and over-the-counter drugs like aspirin and ibuprofen (some brands: Advil. These babies are usually kept in the hospital for several days for observation (watching to make sure they are okay) before they go can home. A baby born to a mother who has ITP may have a low blood platelet count a few days to a few weeks after birth. Another option includes filtering antibodies from the blood. If the baby's platelet count is very low. Some people with certain blood types may have anti-RhD therapy. such as rituximab. which is a shot or series of shots that also work to reduce antibodies in the blood. About 5% of women have mildly low platelet counts at the end of a normal pregnancy. you should avoid medicines that increase risks for bleeding. The platelet count goes back to normal right after delivery. You should also limit alcohol because it can decrease the ability of your blood to clot. because platelet counts may be low for other reasons. Return to top Idiopathic thrombocytopenic purpura (ITP) Definition By Mayo Clinic staff . Return to top What can I do if I have ITP? If you have ITP. treatment is available to speed recovery. Motrin). The cause of this is unknown.

also called immune thrombocytopenic purpura. with surgery. In adults. however. treatment for idiopathic thrombocytopenic purpura usually isn't necessary. Symptoms By Mayo Clinic staff CLICK TO ENLARGE Petechiae Idiopathic thrombocytopenic purpura (ITP) may have no symptoms. but this shouldn't be confused with ITP Superficial bleeding into your skin that appears as a rash of pinpoint-sized reddish-purple spots (petechiae). the disorder is often chronic. Children often develop idiopathic thrombocytopenic purpura after a viral infection and usually recover fully without treatment.Idiopathic thrombocytopenic purpura (ITP). More serious cases may be treated with medications or. ITP results from unusually low levels of platelets the cells that help your blood clot. in critical situations. Idiopathic thrombocytopenic purpura affects both children and adults. If you don't have signs of bleeding and your platelet count isn't too low. usually on your lower legs Prolonged bleeding from cuts Spontaneous bleeding from your gums or nose Blood in urine or stools Unusually heavy menstrual flows . Treatment of idiopathic thrombocytopenic purpura depends on your symptoms and platelet count. When signs and symptoms do occur. is a bloodclotting disorder that can lead to easy or excessive bruising and bleeding. they may include: Easy or excessive bruising (purpura) your skin naturally bruises and bleeds more easily as you age.

000. As the number of platelets decreases. see your doctor. or develops a rash of pinpoint-sized red spots. Causes By Mayo Clinic staff The exact cause of ITP isn't known. however. such as the mumps or the flu." It is known. That's why it's referred to as idiopathic. marking the platelets for destruction. The spleen. triggering it to malfunction. It's also important to seek medical advice if you're a woman and suddenly develop significantly increased menstrual bleeding. which means "of unknown cause. internal bleeding may occur despite a lack of any injury.Profuse bleeding during surgery When to see a doctor If you or your child has abnormal bleeding or bruising. Ordinarily. The greatest risk is when your platelet count falls very low below 10. as this may be a sign of ITP. Risk factors By Mayo Clinic staff .000 platelets per microliter. Serious or widespread bleeding indicates an emergency and requires immediate care.000 platelets per microliter of circulating blood. the immune system malfunctions and begins attacking platelets as if they were foreign substances. At this point. Antibodies produced by your immune system attach themselves to the platelets. your risk of bleeding increases. It may be that an infection sets off the immune system. that in people with idiopathic thrombocytopenic purpura. Adults and children with ITP often have platelet counts below 20. the disorder follows a viral illness. In most children with ITP. which helps your body fight infection. The result of this case of mistaken identity is a lower number of circulating platelets than normal. you have anywhere from 150.000 to 450. recognizes the antibodies and removes the platelets from your system.

Age. also makes you permanently more vulnerable to infection. In fact. Complications are more likely to arise from the treatments corticosteroids and surgery used for chronic or severe ITP. which may be performed if corticosteroids aren't working. Long-term use of corticosteroids can cause serious side effects. ITP is actually far more common in people older than 60 than it is in younger adults. although the risk of an overwhelming infection in a healthy person who has had a splenectomy is low. such as mumps. Pregnancy . including: Osteoporosis Cataracts Loss of muscle mass Increased risk of infection High blood sugar. Major bleeding is rare with ITP. Recent viral infection. ITP clears on its own within two to eight weeks. even diabetes Removal of your spleen (splenectomy). which can be fatal.ITP is a fairly common blood disorder and can occur in anyone at almost any age. measles or a respiratory infection. but these factors increase your risk: Your sex. In most children. Women are about twice as likely to develop ITP as men are. Many children with ITP develop the disorder after a viral illness. Complications By Mayo Clinic staff The biggest risk associated with idiopathic thrombocytopenic purpura is bleeding. Once considered a young person's disease. however. especially bleeding into the brain (intracranial hemorrhage). many therapies pose more serious potential risks than does the disease.

Even better. Take along a family member or friend. you and your doctor may discuss treatment to maintain a stable platelet count. can be brief. you are likely to have further blood tests that require drawing a small amount of blood from a vein in your arm. Include key personal information. so it can help to be prepared. In some cases. It can be difficult to absorb all the information provided during an appointment. though antibodies to platelets can cross the placenta and affect the baby's platelet count. take the original bottles and a written list of the dosages and directions. Here are some tips to help you get ready for your appointment: What you can do Write down all your symptoms even those that seem unrelated to your current problem. The person who accompanies you may remember something that you forgot or missed. It's likely that your baby's platelet count will improve without treatment. If this happens. including vitamins. You are also likely to be referred to a doctor who specializes in blood disorders (hematologist) for further evaluation and treatment. even with specialists. taking into account the effects on your baby. If you're pregnant and your platelet count is very low or you have bleeding. your baby's doctor will want to monitor your child for several days. Make a list of all medications. and there's often a lot of ground to cover. that you're taking. you have a greater risk of heavy bleeding during delivery. . because your baby's platelet count may drop before it starts to rise. but if the count is very low. such as major stresses or recent life changes.Pregnant women with mild ITP usually have a normal pregnancy and delivery. a baby may be born with a low number of platelets. In such cases. Preparing for your appointment By Mayo Clinic staff Because a low platelet count may not cause symptoms. If your doctor thinks you might have ITP. treatment can help speed recovery. Appointments. the problem is often discovered when you have a blood test for another reason. herbs and over-the-counter drugs.

This test is often used to confirm the number of platelets observed in a complete blood count. so I can learn more about this condition? Tests and diagnosis By Mayo Clinic staff Doctors usually diagnose idiopathic thrombocytopenic purpura by excluding other possible causes of bleeding and a low platelet count. A sample of your blood is placed on a slide and observed under a microscope. such as an underlying illness or medications you may be taking. then a diagnosis of ITP may be made. and will ask you about previous illnesses you've had and the types of medications and supplements that you've recently taken. your doctor may need a: Physical exam. To diagnose ITP. If you run out of time. Questions you may want to ask include: What tests are needed to confirm the diagnosis? Is this condition temporary or long-lasting? What treatments are available and what do you recommend? What will happen if I do nothing? What are the possible side effects of the treatments you're suggesting? How can I avoid those side effects? Can you refer me to a website or other source. . Your doctor will look for signs of bleeding under your skin. Complete blood count. but the platelet count is low. Start with the problems that concern you most. With ITP. This common blood test is used to determine the number of white and red blood cells and platelets in a sample of your blood. If no other underlying problem is causing your signs and symptoms. including a complete medical history. Blood smear. ask to speak with a nurse or physician's assistant or leave a message for your doctor. white and red blood cell counts are usually normal. Don't be afraid to ask questions or to speak up when you don't understand something your doctor says.Write down questions for your doctor.

even years later. complete recovery may still occur. The first line of therapy for ITP is a corticosteroid. Adults with mild cases of ITP may require nothing more than regular monitoring and platelet checks. Another test that may help identify the cause of a low platelet count is a bone marrow exam. Your doctor may also have you discontinue certain drugs that can further inhibit platelet function. idiopathic thrombocytopenic purpura usually runs its course without the need for treatment. which removes the liquid portion of your marrow. In some cases. your bone marrow will be normal because your low platelet count is caused by the destruction of platelets in your bloodstream and spleen not by a problem with the bone marrow. ibuprofen (Advil. both procedures are performed at the same time (bone marrow exam). Both the liquid and solid bone marrow samples are frequently taken from the same place on the back of one of your hipbones. In many cases. which can help raise your platelet count by decreasing the activity of your immune system. spongy tissue in the center of your large bones. In children. a sample of solid bone marrow is removed in a procedure called a bone marrow biopsy. Treatment usually consists of medications and sometimes surgery (splenectomy). A needle is inserted into the bone through an incision. others) and the blood-thinning medication warfarin (Coumadin). Once your platelet count is . Or. If you have ITP. About 80 percent of children with idiopathic thrombocytopenic purpura recover completely within six months. you and your doctor may opt for treatment. Platelets are produced in your bone marrow soft.Bone marrow examination. But if your symptoms are troublesome and your platelet count remains low. you may have a bone marrow aspiration. usually prednisone. Treatments and drugs By Mayo Clinic staff The goal of treating ITP is to ensure a safe platelet count and prevent bleeding complications while minimizing treatment side effects. Medications Common medications used to treat idiopathic thrombocytopenic purpura include: Corticosteroids. Even in children who develop chronic ITP. Motrin. such as aspirin.

Surgery If you have severe ITP and an initial course of prednisone hasn't helped. which helps prevent bruising and bleeding. but long-term use of these medications isn't recommended because of the risk of serious side effects.back to a safe level. regardless of age or platelet count. you can gradually discontinue taking the drug under the direction of your doctor. These drugs help your bone marrow produce more platelets. this takes about two to six weeks. severe bleeding can occur with ITP. nausea and vomiting. increased risk of infections and loss of calcium from your bones (osteoporosis). your doctor will likely recommend that you take calcium and vitamin D supplements to help maintain your bone density. . such as immune globulin. The problem is that many adults experience a relapse after discontinuing corticosteroids. A new course of corticosteroids may be pursued. In general. If you have critical bleeding or need to quickly increase your blood count before surgery. some people relapse even after splenectomy. however. You and your doctor will want to weigh the benefits of the medication against these risks. Serious post-surgical complications sometimes occur. Intravenous immune globulin (IVIG). Possible side effects include headache. but the effect usually wears off in a couple of weeks. surgical removal of your spleen (splenectomy) may be an option. joint or muscle pain. given intravenously. The newest medications approved to treat ITP are romiplostim (Nplate) and eltrombopag (Promacta). nausea or vomiting. dizziness. These medications are quick and effective. What's more. Possible side effects include headache. Emergency treatment Although rare. If you've taken corticosteroids for longer than three months. and an increased risk of blood clots. This eliminates the main source of platelet destruction in your body and improves your platelet count within a few weeks. Thrombopoietin receptor agonists. and not having a spleen permanently increases your susceptibility to infection. you may receive medications. Splenectomy for ITP is not as routinely performed as it once was. Severe or widespread bleeding is life-threatening and demands emergency care. This usually includes transfusions of platelet concentrates. including cataracts. Splenectomy is rarely performed in children because of their high rate of spontaneous remission. intravenous methylprednisolone (a type of corticosteroid) and intravenous immune globulin. high blood sugar.

Treatment decisions are usually based on: Severity of signs and symptoms (active bleeding is usually an indication for treatment) Platelet count even relatively low counts (less than 30. questions about long-term side effects remain. peptic ulcer) or needed medications. and their effectiveness has yet to be proved. Azasan). usually at the lowest effective dose. infections.000 platelets per microliter of blood) may not merit treatment. pylori treatment. New medications that increase platelet production. have been used to treat ITP. alcoholism. especially if you have no active bleeding and have a fairly sedentary lifestyle Your age and willingness to undergo treatment Risk of bleeding relative to lifestyle. Eliminating the bacteria has helped increase platelet count in some people. Other possible treatments include: Immunosuppressant drugs. Because of the potential complications of both the disease and its treatment. such as rituximab (Rituxan) the most commonly used of this group cyclophosphamide (Cytoxan) and azathioprine (Imuran. but they can cause significant side effects. but the results for this therapy are inconsistent and need to be studied further. such as participation in sports or other vigorous physical activities that may predispose you to injury Risk of bleeding based on other medical conditions (high blood pressure. your doctor may recommend another course of corticosteroids. and relapse is possible when the drugs are stopped. chronic liver disease. H. especially eltrombopag and AMG 531.Other treatments If neither the initial round of corticosteroids nor a splenectomy has helped you achieve remission and your symptoms are severe. such as aspirin . some people find that the side effects of treatment are more burdensome than the effects of the disease itself. Some people with ITP are also infected with Helicobacter pylori. are being studied in clinical trials. Experimental drugs. it's important for you and your doctor to carefully weigh the benefits and risks of treatment. Medications that suppress the immune system. Although they appear to be well tolerated. For example. the same bacteria that cause most peptic ulcers.

Infection in someone who's had a splenectomy may be more severe. since studies show that most patients have either normal or diminished platelet production. ITP is a decrease in the number of circulating platelets in the absence of toxic exposure or a disease associated with a low platelet count. Pathophysiology ITP is primarily a disease of increased peripheral platelet destruction. Limit alcohol. the following steps may help control your risk of bleeding and other complications: Avoid platelet-impairing medications. . including fever. and seek prompt treatment. also known as primary immune thrombocytopenic purpura and autoimmune thrombocytopenic purpura. Introduction Background Idiopathic thrombocytopenic purpura (ITP). Motrin. Relative marrow failure may contribute to this condition. If you've had your spleen removed.Potential side effects of ITP therapies Lifestyle and home remedies By Mayo Clinic staff If you have idiopathic thrombocytopenic purpura. Over-the-counter drugs. with most patients having antibodies to specific platelet membrane glycoproteins. The 2 distinct clinical syndromes manifest as an acute condition in children and a chronic condition in adults. Watch for signs of infection. can impair platelet function. Your doctor may recommend avoiding competitive sports or other activities that might increase the risk of injury and bleeding. Choose low-impact physical activities. be alert for any signs of infection. others). such as aspirin and ibuprofen (Advil. is defined as isolated thrombocytopenia with normal bone marrow and the absence of other causes of thrombocytopenia. Excessive alcohol intake can adversely affect blood clotting. last longer and have more serious implications than in someone who still has an intact spleen.

Chronic ITP persists longer than 6 months without a specific cause. Mortality/Morbidity Hemorrhage represents the most serious complication. A study in Kuwait reported a higher incidence of 125 cases per 1. An average estimate of the incidence in children is 50 cases per 1.000 per year.000. More than 72% of patients older than 10 years are female. In acute ITP (children). In patients with severe thrombocytopenia. distribution is equal between males (52%) and females (48%). Sex In chronic ITP (adults). The mortality rate from hemorrhage is approximately 1% in children and 5% in adults. intracranial hemorrhage is the most significant.000.000 per year. International According to studies in Denmark and England. respectively. Frequency United States The incidence of ITP in adults is approximately 66 cases per 1. Spontaneous remission occurs in more than 80% of cases in children but is uncommon in adults.6:1. Older age and previous history of hemorrhage increase the risk of severe bleeding in adult ITP.000.000. . 47.Acute ITP often follows an acute infection and has a spontaneous resolution within 2 months. New cases of chronic refractory ITP comprise approximately 10 cases per 1. predicted 5-year mortality rates from bleeding are significantly raised in patients older than 60 years versus patients younger than 40 years.2%.000 per year.8% versus 2.000.000 per year. the female-to-male ratio is 2. childhood ITP occurs in approximately 10-40 cases per 1.000 per year.

heparin. as these may determine the aggressiveness of management. One study used 3 distinct methods to document drugs that may be associated with drug-induced immune thrombocytopenia (DITP). and precipitating factors include the following: Abrupt onset (childhood ITP) Gradual onset (adult ITP) Purpura Menorrhagia Epistaxis Gingival bleeding Recent live virus immunization (childhood ITP) Recent viral illness (childhood ITP) Bruising tendency Limited data are available on the recurrent form of the disease. Clinical History Focus on the symptoms of bleeding (eg. Address risk factors for increased bleeding. alcohol. Medications can be a common etiology for inducing thrombocytopenia. sulfonamides) that may cause thrombocytopenia. or active lifestyle. such as GI disease. Common signs. Peak prevalence occurs in children aged 2-4 years. and patients should have their medications carefully reviewed.Age Peak prevalence occurs in adults aged 20-50 years. type. Though one third of patients had . urologic disease. Elicit risk factors for HIV and systemic symptoms linked to other illnesses or to medications (eg. 24 drugs had evidence of causing thrombocytopenia by all 3 methods. quinidine/quinine. but.2 Approximately 1500 drugs are associated with thrombocytopenia. duration) and on symptoms that may exclude other causes of thrombocytopenia. CNS disease. using this analysis. severity. One study showed a 6% prevalence of recurrent ITP with most patients (69%) having only one recurrence. symptoms. Approximately 40% of all patients are younger than 10 years.1.

menorrhagia Retinal hemorrhages Evidence of intracranial hemorrhage. Causes Immunoglobulin G (IgG) autoantibodies on the platelet surface Differential Diagnoses Disseminated Intravascular Coagulation HIV Infection and AIDS Thrombocytopenic Purpura . arthritis). 12% in children). thrombosis.000/mm3. nephritis. Spontaneous bleeding when platelet count is less than 20.their recurrent episode within 3 months of their initial one. with possible neurologic symptoms Nonpalpable spleen: The prevalence of palpable spleen in patients with ITP is approximately the same as that in the non-ITP population (ie. autoimmune diseases (eg. 3% in adults. cutaneous vasculitis. which mostly occur in dependent regions Hemorrhagic bullae on mucous membranes Purpura Gingival bleeding Signs of GI bleeding Menometrorrhagia. Common physical findings include the following: Nonpalpablepetechiae. and infection. particularly HIV. Physical Evaluate the type and the severity of bleeding and try to exclude other causes of bleeding. the remainder of patients had at least a 3month interval between episodes. Seek evidence of liver disease.

quinine/quinidine. The WBC count and hemoglobin typically are normal. autoimmune. sulfonamides) Infection/sepsis Acute leukemia Myelodysplastic syndrome Malignancy Megaloblastic anemia Isoimmune neonatal purpura Transfusion Factitious Workup Laboratory Studies CBC Isolated thrombocytopenia is the key finding regarding laboratory evaluation. unless severe hemorrhage has occurred. Coagulation studies are normal. or infectious diseases Pregnancy-associated thrombocytopenia Drug-induced immune thrombocytopenia (alcohol. heparin. and a bleeding time is not useful. . Truly giant platelets on peripheral smear suggest congenital thrombocytopenia.Other Problems to Be Considered Pseudothrombocytopenia (platelet clumping in the presence of ethylenediaminetetraacetic acid [EDTA]) Liver disease Myelodysplasia Lymphoproliferative.

In patients without life-threatening complications. are appropriate. if possible. or 1 U/10 kg 1 U of platelets to increase count of a 70-kg adult by 5-10. A consultation with a hematologist may be required to make a decision regarding the transfusion of platelets. Consultations . Send a blood specimen to the lab for type and screen in case platelet transfusion is necessary. focus ED care on confirming the diagnosis. Emergent splenectomy is indicated in patients with life-threatening bleeding in whom medical therapy fails. Most patients with undiagnosed thrombocytopenia and purpura will need admission for further evaluation and treatment. and initiating intravenous (IV) fluids to maintain hemodynamic stability. with or without platelet transfusions. and initiating therapy as needed. In the patient with known ITP. since ITP is a diagnosis of exclusion. Prehospital airway control may be necessary for a large intracranial hemorrhage.000/mm3 Splenectomy is reserved for patients in whom medical therapy fails. EMS providers should be aware of the potential for serious bleeding complications in patients with idiopathic thrombocytopenic purpura (ITP). Treatment Prehospital Care Prehospital care focuses on the ABCs. which include providing oxygen. Guidelines for transfusion dosage 6-8 U of platelet concentrate.Imaging Studies A CT scan of the head is warranted if concern exists regarding intracranial hemorrhage. high-dose parenteral glucocorticoids and IV immunoglobulin (IVIg). controlling severe hemorrhage. Emergency Department Care Life-threatening bleeding requires conventional critical care interventions. Platelet transfusion is indicated for controlling severe hemorrhage.000/mm3 and an 18-kg child by 20. Platelet survival is increased if the platelets are transfused immediately after IVIg infusion.

and the degree of symptoms. Treatment also is indicated for those adults with risk factors for bleeding (eg. risks include worsening immunosuppression and infection or sepsis. Consultation with a hematologist may be needed prior to starting therapy. if appropriate. However.Consult a hematologist for assistance in confirming the diagnosis or. weekly methylprednisolone. Although generally less toxic than IV steroids. For immunosuppressive therapy and splenectomy. recent studies using higher dosages of IV RhIG in acute ITP in children and adults show platelet count increases at 24 hours faster than medicating with steroids and at 72 hours similar to IVIg. loss of muscle mass. also known as IV Rh immune globulin (IG). with an estimated incidence of 1 in 1115 patients. Children who have platelet counts <20. was not recommended by the 1996 American Society of Hematology practice guidelines. vigorous lifestyle) and in patients with a platelet count <20. dosage. using a combination of weekly vincristine. This therapy is not appropriate for patients who have undergone splenectomy. Some small studies have shown limited success. According to one study3 .000-30. Treatment is indicated for adults with counts <50.000/mm3 do not require treatment.000/mm3 with significant mucous membrane bleeding. hypertension. Acute intravascular hemolysis after infusing IV RhIG has been reported. and an increased risk of infection. Steroid use and immunosuppressives and splenectomy may be undesirable because of their associated complications. glaucoma.000/mm3 and are asymptomatic or have only minor purpura do not require routine treatment.000/mm3. Indications for use. this includes osteoporosis. Consultation by other surgical specialists may be required for extensive hemorrhage at other sites. Medication Glucocorticoids and IVIg are the mainstays of medical therapy. Studies of the use of multiagent therapies in refractory patients are ongoing. Children who have platelet counts >30. IV RhIG is more expensive than IV steroids. IV anti-(Rh)D. For long-term steroid use.000/mm3 and minor purpura should receive specific treatment. Studies in children with chronic ITP show that escalating or elevated doses of IV RhIG have comparable responses to those of high-dose IVIg therapy in children. arranging disposition and follow-up care. the absolute platelet count. and route of administration are based on the patient's clinical condition. in the patient with known ITP. both until platelet . Consult a neurosurgeon for intracranial hemorrhage. peptic ulcer disease. cataracts.000/mm3 and significant mucous membrane bleeding and those who have platelet counts <10. Adults with platelet counts >50.

may not need therapy. DOC for all adult patients with platelet counts <50. glucocorticoids. splenectomy). or patients with counts 30. While they show promise for raising platelet counts. It is unlikely that emergency physicians should be prescribing these agents without being under the recommendation of a hematologist. such as thrombopoietin (TPO) receptor-binding agents. IVIg immunoglobulin.7 Both of these agents require registration in a database. withholding medical therapy may be appropriate for asymptomatic patients. regardless of count. They have been shown to increase platelet count in ITP. and splenic radiation have been studied. such as cyclophosphamide.000/mm3. they serve as additional therapeutic measures in ITP refractory-toprimary therapy (eg. Sterapred) Useful in treating inflammatory and allergic reactions.4. there are potential safety concerns such as thrombocytosis and rebound thrombocytopenia. Asymptomatic patients with platelet counts >20.000/mm3 with only minor purpura. Other therapies.6. though larger prospective studies are needed. may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Newer studies on rituximab suggest that this agent is an effective treatment option in splenectomized refractory or relapsed ITP patients. however. are available to patients with chronic ITP who have failed other therapies.000/mm3. and cyclosporine orally twice daily until the platelet count is normal for 36 months seems promising.000/mm3. vinca alkaloids. Orasone.5 Clinical trials have shown promise for agents that directly stimulate platelet production.000-50. interferon alfa. danazol. Many case series discussing these treatments are too small to show sufficient evidence of a clinically significant reduction in bleeding or mortality rate. Glucocorticoids These agents are used to treat idiopathic and acquired autoimmune disorders. dapsone.counts reached 50. accessory splenectomy. . eltrombopag and romiplostim. Two new agents. azathioprine. Prednisone (Deltasone.

hypokalemia. abrupt discontinuation may cause adrenal crisis Methylprednisolone (Solu-Medrol. or tubercular skin infections DosingInteractionsContraindicationsPrecautionsPregnancyB . psychosis. osteoporosis. viral. and growth suppression. and rifampin may increase metabolism (consider increasing maintenance dose). monitor for hypokalemia with coadministration of diuretics DosingInteractionsContraindicationsPrecautionsDocumented hypersensitivity. phenobarbital.000/mm3 DosingInteractionsContraindicationsPrecautionsEstrogens may decrease clearance. myopathy.Fetal risk not confirmed in studies in humans but has been shown in some studies in animals PrecautionsMay cause severe infections. or for all patients with platelet counts <30. phenytoin. Depo-Medrol) Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased permeability. edema. DosingInteractionsContraindicationsPrecautionsAdult Loading dose: 125-250 mg IV . peptic ulcer disease.000/mm3. fungal.000/mm3. Used as alternative glucocorticoid of choice for all patients with severe. myasthenia gravis. hyperglycemia. Careful observation without medical treatment may be appropriate in some asymptomatic children. concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia.DosingInteractionsContraindicationsPrecautionsAdult 1-2 mg/kg/d PO Pediatric 4-8 mg/kg/d PO for severe. lifethreatening bleeding or children with platelet counts <30. osteonecrosis. life-threatening bleeding with platelet counts <50. euphoria.

hypokalemia. DosingInteractionsContraindicationsPrecautionsAdult . or tubercular skin infections DosingInteractionsContraindicationsPrecautionsPregnancyC . may use if benefits outweigh risk to fetus PrecautionsHyperglycemia.000/mm3 with minor purpura. can be used alone or in addition to glucocorticoid therapy. monitor patients for hypokalemia when taking concurrent diuretics DosingInteractionsContraindicationsPrecautionsDocumented hypersensitivity.5-1 mg/kg/dose IV q6h for up to 5 d Pediatric Loading dose: 2 mg/kg IV Maintenance dose: 0. myopathy. estrogens may increase levels. peptic ulcer disease. edema. psychosis.Maintenance dose: 0. Intravenous immune globulin (IVIg) DOC for severe.5-1 mg/kg/dose IV q6h for up to 5 d DosingInteractionsContraindicationsPrecautionsCoadministration with digoxin may increase digitalis toxicity secondary to hypokalemia. osteonecrosis. temporary rise in platelet count. osteoporosis. viral. and rifampin may decrease levels (adjust dose). phenytoin.Fetal risk revealed in studies in animals but not established or not studied in humans. life-threatening bleeding or for children with platelet counts <20. euphoria. fungal. Consider IVIg if the situation requires a rapid. and infections are possible complications Blood products Administration of IVIg may temporarily increase platelet counts in some children and adults with ITP. growth suppression. phenobarbital.

Fetal risk revealed in studies in animals but not established or not studied in humans. may increase serum viscosity and thromboembolic events. Interacts with transmembrane domain of human TPO receptor and induces megakaryocyte proliferation and differentiation from bone marrow progenitor cells. may increase risk of migraine attacks. 6-fold increase in ESR for 2-3 wk. pruritus. IgA deficiency and antiIgE/IgG antibodies DosingInteractionsContraindicationsPrecautionsPregnancyC .1-2 g/kg IV administered over 1-5 d Pediatric 1 g/kg once DosingInteractionsContraindicationsPrecautionsNone reported DosingInteractionsContraindicationsPrecautionsDocumented hypersensitivity. aseptic meningitis (10%). eg. or petechiae (2-30 d postinfusion) Increases risk of renal tubular necrosis in elderly patients and in patients with diabetes. or preexisting kidney disease. Gammagard S/D). Indicated for thrombocytopenia associated with chronic idiopathic thrombocytopenic purpura in . changes in lab findings associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo. urticaria. volume depletion. and apparent hyponatremia Thrombopoietic Agent These agents directly stimulates bone marrow platelet production.8 Eltrombopag (Promacta) Oral thrombopoietin (TPO) receptor agonist. may use if benefits outweigh risk to fetus PrecautionsCheck serum IgA before IVIg (use IgA-depleted product.

benzylpenicillin. Program phone number is (877) 9-PROMACTA (877-977-6622). UGT1A4. and UGT2B15 inhibitor Coadministration with moderate or strong CYP1A2 (eg. fluvoxamine) or CYP2C8 (eg. CYP2C8. atorvastatin. not to exceed 75 mg/d. Only available through restricted distribution program. gemfibrozil. acetaminophen. selenium. rifampin) Inhibits UGT1A1. UGT1A4. aluminum. DosingInteractionsContraindicationsPrecautionsAdult 50 mg PO qd 1 h ac or 2 h pc East Asian ancestry or moderate-to-severe hepatic insufficiency: 25 mg PO qd Use lowest dose to achieve and maintain platelet count 50 X 109/L to reduce risk of bleeding. antacids. OATP1B1 inhibitor. repaglinide. narcotic. UGT1A1. pravastatin. or supplements containing polyvalent cations (eg. methotrexate. UGT1A1. UGT1A3. NSAID) Chelates polyvalent cations. UGT1A9. calcium-rich foods. UGT2B7. nateglinide.patients experiencing inadequate response to corticosteroids. Not for use to normalize platelet counts but used when clinical condition increases bleeding risk. or splenectomy. and UGT2B15 enzymes and therefore may increase systemic exposure of substrates (eg. UGT2B7. ciprofloxacin. UGT1A3. UGT1A9. magnesium. calcium. immunoglobulins. zinc) . fluvastatin. trimethoprim) inhibitors may inhibit eltrombopag's oxidative metabolism and increase toxicity Coadministration with UGT1A1 or UGT1A3 inhibitors or inducers may affect glucuronidation of eltrombopag Inhibits OATP1B1 and may increase exposure to OATP1B1 substrates (eg. UGT1A6. allow 4-h interval for administration of other medications. rosuvastatin. iron. and UGT1A3 substrate. discontinue if platelet count not increased after 4 wk at maximum dose or if platelet count increases substantially Pediatric Not established DosingInteractionsContraindicationsPrecautionsCYP1A2. UGT1A6. Prescribers must enroll in Promacta Cares program.

not to exceed 10 mcg/kg/wk If platelet count not adequate to control bleeding after 4 wk at maximum dose. monitor CBC count weekly during dose adjustment. and discontinue if levels increase. a mechanism similar to endogenous TPO. a program designed to promote informed risk-benefit decisions before initiating treatment. excessive dose may increase platelet counts and produce thrombotic/thromboembolic complications (discontinue if platelet count >400 X 109/L after 2 wk at lowest dose). may increase risk for hematological malignancies.nplate. adjust in increments of 1 mcg/kg SC qwk to achieve platelet count of 50 X 109/L or greater (median dose in clinical trials was 2 mcg/kg). For more information. DosingInteractionsContraindicationsPrecautionsAdult 1 mcg/kg (actual body weight) SC initially. monthly following stable dose.com or call (877) NPLATE1 (877-675-2831). and at least 4 wk after discontinuation Romiplostim (Nplate) An Fc-peptide fusion protein (peptibody) that increases platelet production through binding and activation of the thrombopoietin (TPO) receptor. and bilirubin.DosingInteractionsContraindicationsPrecautionsNone known DosingInteractionsContraindicationsPrecautionsPregnancyC . Only available through the Nplate NEXUS (Network of Experts Understanding and Supporting Nplate) program. see www. or splenectomy. may cause bone marrow fibrosis because of reticulin fiber deposition.Indicated for chronic immune (idiopathic) thrombocytopenic purpura in patients who have had an insufficient response to corticosteroids. immunoglobulins. AST. discontinue and continue monitoring platelet count for 2 wk Pediatric <18 years: Not established Follow-up . may use if benefits outweigh risk to fetus PrecautionsMay cause hepatic impairment.Fetal risk revealed in studies in animals but not established or not studied in humans. monitor ALT.

steroids. Transfer Transfer may be necessary under the following conditions: A hematologist is not available. Observe for life-threatening bleeding. Elective splenectomy may be necessary if medical therapy fails. platelet transfusion) may be indicated. IVIg. A higher level of intensive care is needed. Blood bank support is insufficient. Further Outpatient Care Close follow-up care with a hematologist is required. Complications Complications of idiopathic thrombocytopenic purpura may include the following: Intracranial or other major hemorrhage Severe blood loss Adverse effects of corticosteroids Pneumococcal infections if the patient must have a splenectomy Prognosis Children Approximately 83% of children have a spontaneous remission. .Further Inpatient Care Rule out other potential causes of thrombocytopenia. and 89% of children eventually recover. Consult with a hematologist. as further treatments (eg. Emergency splenectomy may be necessary if severe bleeding complications due to thrombocytopenia do not respond to medical therapy.

Adults Only 2% of adults have a spontaneous recovery. Miscellaneous Medicolegal Pitfalls Failure to consider other causes of thrombocytopenia Failure to initiate therapy Special Concerns Pregnancy Gestational thrombocytopenia and thrombocytopenia due to preeclampsia are more common than ITP in pregnancy. The pregnant patient with ITP is treated the same as other patients with ITP.More than 50% of patients recover within 4-8 weeks. Concerns about thrombocytopenia increase as term approaches. Because of the increased risk of bleeding. instruct patients to avoid aspirin products. and 5% of patients die from hemorrhage. percutaneous umbilical blood sampling. Approximately 30% of patients have chronic disease. however. and it does not exacerbate a preexisting disease. Emphasize close outpatient follow-up care. Approximately 2% of patients die. and the risks of thrombocytopenia to the newborn must be considered. Patient Education Instruct patients to return for follow-up in order to assess for a potentially reduced platelet count. and IVIg therapy. This is because antiplatelet IgG antibodies can cross the placenta and can induce fetal thrombocytopenia. prednisone. Geriatrics: Patients older than 60 years may be at greater risk of severe bleeding than younger patients (<40 y) at equivalent platelet counts. Pregnancy does not increase the incidence of ITP. . The perinatologist and the hematologist make the ultimate decisions regarding cesarean section (for protection of the newborn against intracranial hemorrhage). approximately 64% of adults eventually recover.

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