What is Immune Thrombocytopenic Purpura (ITP)?
Immune thrombocytopenic purpura ITP Diagnosis. In autoimmune disorders your body makes proteins called antibodies which damage another part of your body. In Immune Thrombocytopenic Purpura (ITP) the antibodies are made against platelets. Once the antibodies have attached to platelets, the platelets do not work so well. They are also removed more quickly by the spleen because they are abnormal.
Thalassemia symptoms depend on the type of thalassemia you have and how severe the anemia becomes. Some people have little or even no symptoms. Other people have mild to severe symptoms. Symptoms of thalassemia may include one or more of the following:
Common symptoms of white blood cell disorders are:
- It is called 'immune' because it is now known that a problem with the immune system is the cause
- Thrombocytopenic, or thrombocytopenia, means not having enough platelets
- Purpura is a purple-red rash. It is caused by tiny bleeds under the skin
- ITP is quite different in children and adults and should be considered separately
Understanding platelets:
Platelets are tiny components of the blood which help blood to clot when we injure ourselves. They are also known as thrombocytes. They are made inside bone, in the bone marrow. They are released into the bloodstream and travel through the body for about seven days, before they are removed by the spleen. The spleen is an organ that lies at the top of the tummy (abdomen) under the ribs on the left-hand side. A normal number of platelets is between 1,50,000 and 4,50,000. This is found by a blood test. If you have too many platelets, your blood will clot too easily. If you do not have enough platelets, you may bruise and bleed more easily than usual immune thrombocytopenic purpura in children.
How common is ITP in children?
ITP occurs in about 2 to 5 of every 100,000 children. It is most common in children around the age of 5 or 6 years, but it can occur at any age.
What are the symptoms?
Common symptoms of white blood cell disorders are:
- Most children will not have any symptoms.
- Those who do develop symptoms have bruising or a purple or red rash - purpura. There may be tiny red spots, or larger areas of bruising or rash. This usually appears over 1-2 days.
- Some have nosebleeds. Older girls may have heavier periods.
- The condition often occurs about 2-3 weeks after an infection (0ften a common viral infection). Occasionally, it follows immunisation. The symptoms often disappear over 6-8 weeks, and by six months in most cases.
- Very occasionally, it causes severe bleeding which requires emergency treatment.
- The platelet levels in about 1 or 2 in 10 affected children do not return to normal after a year. This is then called chronic ITP, which means it is persistent. However, many of these children will never need any treatment.
So, what seems to happen in children with ITP is that the immune system is triggered to produce antibodies against the platelets by an infecting virus or other germ. In most cases, this is a temporary immune reaction that lasts several weeks only, and then symptoms go. But, in a few cases, the immune system continues to be faulty and the condition becomes long-standing.
How is ITP diagnosed?
ITP Diagnosis by a blood test called a complete blood count(CBC). This test shows that there are fewer platelets than normal. The laboratory will also have a look at the blood under a microscope.
Sometimes different tests will be needed to make sure that the low number of platelets is not due to another of the possible causes. Rarely, this involves taking a sample of bone marrow. This is not needed if the blood count and symptoms are typical of ITP.
What is the treatment?
Most children will not need ITP Treatment, even if the number of platelets is very low. The decision to treat is usually based on whether your child has serious bleeding or very pronounced bruising and purpura. This is because some children can have very low platelet counts but still not have any bleeding problems. If the symptoms are mild, usually there will be no need for treatment. Your child may need to have the full blood count repeated on a few occasions to check that the platelet numbers are stable and that the rest of the blood cell counts are remaining normal. If your child has bleeding and more severe bruising or purpura, treatment may be considered. The aim of treatment is to improve symptoms and increase the number of platelets.
If treatment is needed then the decision on what ITP Treatment to use can be difficult. This is because there have not been many studies testing the treatments against each other. The options for treatment include:
Common symptoms of white blood cell disorders are:
- Prednisolone: This is often the first type of treatment tried. It is a steroid medication and is taken as syrup or tablets. This may be given as a high dose over a short period of time (four days) or as a lower dose for a longer time (two weeks). Prednisolone has been shown to increase the number of platelets quickly in about 3 in 4 children with ITP.
- Other Steroid Options: High-dose methylprednisolone or high-dose dexamethasone. These are other types of steroid medicines that have also been shown to be effective in differing degrees. These are less often used now than they used to be.
- Intravenous Immunoglobulin (IVIg): This is an injection of a protein into the bloodstream and has been shown to work well in about 8 in 10 children in increasing the number of platelets. It is not usually used as the first option because it involves an injection. It can also cause quite a lot of side-effects and is quite expensive. It may be used as an emergency treatment if your child has severe bleeding or needs surgery.
- Anti-D Immunoglobulin: This is another type of protein that is also effective and causes fewer side-effects than IVIg. It can only be given to children whose blood group is rhesus (RhD) positive.
- Platelet Transfusions: In a life-threatening situation your child may be given a transfusion of platelets at the same time as being treated with steroids and IVIg. This only helps to increase the number of platelets for a short time. The transfused platelets are also attacked by the antibody that the body has produced and are destroyed by the spleen.
- Other Medicines: A number of other medicines may be used. Such medicine are Eltrombopag, Romiplostim and Rituximab.
Treatment
Treatment for thalassemia depends on which type of thalassemia you have and the severity of your symptoms. If you have no or only mild symptoms, you may need little or no treatment. Thalassemia Treatment for moderate to severe forms of thalassemia often includes regular blood transfusions and folate supplements. Folate (also called folic acid) helps your body make healthy blood cells. Alpha thalassemia can sometimes be mistaken for low-iron anemia, and iron supplements may be recommended as a treatment. But iron supplements have no effect on thalassemia.
If you have many blood transfusions, too much iron may build up in your blood. If this happens, you will need chelation (say: “key-LAY-shun”) therapy to remove extra iron from your body. You shouldn’t take iron supplements if you receive blood transfusions.
In the most severe cases, bone marrow or stem cell transplants may help by replacing damaged cells with healthy ones from a donor (usually a relative such as a brother or sister).
How can I cope with Thalassemia?
Although you can’t prevent inheriting thalassemia, you can manage the disease so you can have the best quality of life possible. Key steps include:
- Follow your treatment plan get blood transfusions as often as your doctor recommends. Make sure to take your iron chelation medicine and/or folic acid supplements.
- Get ongoing medical care have regular medical checkups and get the medical tests your doctor suggests. These may include tests relating to thalassemia, as well as your overall health. Be sure to get any vaccinations for flu, pneumonia, hepatitis Band meningitis that your doctor recommends.
- Take care of yourself follow a healthy eating plan. Lower your chance of getting an infection by washing your hands often, and avoiding crowds during flu and cold season. Keep the area around your transfusion site clean. Call your doctor if you develop a fever or other signs of infection.
- Look for information and support join a support group or talk with others who have the disease to learn coping strategies. Be sure to discuss any changes in your treatment plan with your doctor.
Complications
What are the complications of thalassemia?
Thalassemia can lead to other health problems:
- An enlarged spleen your spleen helps your body fight infections and filters out damaged blood cells. If you have thalassemia, your spleen may have to work harder than normal, which can cause it to enlarge. If your spleen becomes too large, it may have to be removed.
- People who have thalassemia are more likely to get blood infections, especially if they have a lot of blood transfusions. Some types of infection can be worse if you’ve had your spleen removed.
- Bone problems thalassemia can cause bone deformities in the face and skull. People who have thalassemia may also have severe osteoporosis (brittle bones).
- Too much iron in your blood this can cause damage to the heart, liver, or endocrine system (glands in the body that make hormones, like the thyroid gland and adrenal glands).
What should I do if I’m a carrier of thalassemia and I want to get pregnant?
Some severe types of thalassemia can cause babies to die before they are born or soon after. If you or your partner knows you are a carrier for thalassemia, you may want to talk to your doctor or a genetic counsellor before getting pregnant. Certain tests may be able to show which type of thalassemia you are carrying. Once you are pregnant, prenatal testing can show whether or not your baby has thalassemia.