Idiopathic Thrombocytopenic Purpura
- autoimmune thrombocytopenia
Idiopathic thrombocytopenic purpura, abbreviated as ITP, is an autoimmune disorder that causes very low platelet counts. An autoimmune disorder is one in which a person, for reasons that healthcare professionals often do not understand, produces antibodies against his or her own tissues.
What is going on in the body?
Platelets, which are made by the bone marrow, are essential for blood clotting. In ITP, the body produces antibodies that attack the platelets and destroy them. Normally the body makes antibodies to fight infections.
In ITP, the body for some reason makes antibodies against its own tissues, called autoantibodies. These autoantibodies act to destroy platelets. The result is a low platelet count, known as thrombocytopenia.
The cause of ITP is unknown. But certain viral infections seem to precede some cases of ITP. Antibodies created to fight the infection then turn and attack the platelets, which are then removed from the bloodstream. Normal platelet counts are between 150,000 and 450,000 per cubic ml of blood. People with ITP may have very low platelet counts, often lower than 20,000 to 50,000.
What are the causes and risks of the disease?
Viral infection is believed to be one of the things that causes the body to make antibodies against platelets. A recent immunization using a live virus is also associated with an increased risk of ITP.
ITP is twice as common in females as in males. The disease is most common in adults who are 20 to 50 years of age and in children who are 2 to 9 years of age. ITP is more likely to cause bleeding in older individuals or people who have had bleeding problems in the past.
What can be done to prevent the disease?
There is no known prevention for ITP.
How is the disease diagnosed?
ITP is diagnosed based on symptoms and results of blood tests. A complete blood count, (CBC), identifies low platelet counts. The blood can also be tested for platelet autoantibodies to confirm the diagnosis. If the blood tests do not confirm the diagnosis, a bone marrow biopsy can be performed.
During a bone marrow biopsy, a sample of bone marrow is taken using a large hollow bore needle, usually from a wing of the pelvis or from the sternum (breast bone) and studied. The bone marrow sample should show a normal mixture of marrow cells, including those that develop into platelets. Because ITP involves platelet destruction in the bloodstream, the results of the bone-marrow tests should be normal in ITP. An abnormal test suggests another diagnosis.
Long Term Effects
What are the long-term effects of the disease?
Most cases of ITP do resolve. However, some people develop chronic ITP. They may have prolonged episodes of low platelet counts and the complications that go with them. Other people may have long-term health problems from intracerebral hemorrhage
or gastrointestinal bleeding.
What are the risks to others?
ITP is not contagious and poses no risk to others.
What are the treatments for the disease?
If a person with ITP is hemorrhaging severely, the first treatment will be a transfusion of platelets. In some cases, the platelet count returns to normal on its own. This spontaneous remission of the disease is more common in children with ITP than in adults.
Corticosteroids, such as methylprednisolone or dexamethasone, are given to suppress the immune response. These can be given either intravenously or as tablets to be taken orally. If steroid therapy does not improve the platelet count, more aggressive interventions can be tried. These include using intravenous immunoglobulin (IVIG).
IVIG binds the autoantibodies that are attacking the platelets and removes them from the system. Once they are removed from the bloodstream, they cannot destroy the platelets. This treatment is very expensive and requires intravenous administration of medication.
Another way to remove the autoantibodies causing the disorder is to filter the person's blood using a technique called plasmapheresis. A large catheter is placed into a blood vessel, and the blood is filtered through the plasmapheresis unit - removing the antibodies - and then returned to the circulation. This treatment is usually reserved for severe cases of ITP.
Alternatively, a splenectomy, or removal of the spleen, can be performed. Often this will cure the condition. Other experimental treatments have been tried with varying results.
What are the side effects of the treatments?
Medications used to suppress the immune system may cause allergic reactions or increased risk of infection. Surgery can be complicated by bleeding, infection, or an allergic reaction to the anesthetic.
What happens after treatment for the disease?
Treatment of ITP continues until a normal platelet count is restored. Then, the platelet count is monitored, and treatment can resume if the platelet count begins to fall again.
How is the disease monitored?
ITP is monitored by repeated complete blood counts, or CBCs. Any new or worsening symptoms should be reported to the healthcare professional.