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Idiopathic Thrombocytopenic Purpura
Also Known as ITP
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Idiopathic thrombocytopenic purpura is an immune disease that can lead to excessive bleeding and bruising. Its sufferers have a low level of platelets in their body so their blood does not clot properly. The disease can affect both adults and children. In kids, they seem to get the disease following a viral infection. The disease is then self limited and the child recovers with no treatment. In adults, ITP tends to be a chronic condition.
The symptoms of idiopathic thrombocytopenic purpura can be very mild or absent if you have mild disease and you have no bleeding. If the platelet count is low enough, however, you need to look out for excessive bruising (also called purpura) and excessive bleeding with injury. You can have petechial lesions under the skin, which are pinpoint areas of red spots that occur in clusters, especially on the lower legs. You can have nose bleeds and or gum bleeding. Blood can occur in the urine or in the stools and, if you are female, you can have heavy menstrual bleeding. If you have surgery, you can bleed profusely.
The reasons behind ITP are not clear. This is why it is called an idiopathic disease. It is believed to be related to an autoimmune disease that attacks and destroys the platelets in the body. Antibodies treat the platelets as though they are foreign and destroy the platelets. The spleen is active in the disease by sequestering and destroying the platelets that have been marked for destruction.
The normal platelet count is between 150,000 and 450,000. Your risk of bleeding goes up as the platelet count goes down. Platelets seem to control bleeding fairly well even when the platelet count is low but a platelet count of 10,000 is so low that spontaneous bleeding can occur.
Children who have ITP often have had a recent viral illness, including the flu or respiratory illness. The infection triggers the immune system to malfunction, attacking platelets. Infection has no relationship to adult onset ITP.
Risk factors for ITP include being female, especially being a young female. They are twice as likely as men to develop ITP. ITP is common in young people and in men and women over the age of 60. A recent viral infection is a risk factor in young people.
Complications of ITP include bleeding into the brain, which is life threatening. On the other hand, the treatments of ITP carry a higher risk of complications than the disease itself. For example, the use of corticosteroids in ITP can cause osteoporosis, loss of muscle mass and increased infection risk. Cataracts, too, are possible. The surgery to correct the low platelet count involves a splenectomy or spleen removal. This can be a dangerous surgery because the platelet count is particularly low and bleeding can result. Pregnant women can have complications of pregnancy. The antibodies can cross the placenta and can affect the infant's platelet count. The pregnant woman has a greater chance of bleeding during labor and delivery, which can be severe.
The diagnosis of ITP is not easy. It comes by excluding other things as causes of low platelets and bleeding. Doctors will check a complete blood count, which assesses the platelet count, among other things. A smear of blood on the slide will show an absence of platelets. Bone marrow testing will show an increase in platelet formation to make up for the platelets lost in the spleen. The bone marrow may actually be normal as well.
ITP is not usually treated in children as the condition often passes and is not a problem. A hematologist often treats such conditions by monitoring the platelet count and treating any complications. Treatment in adults can involve cortisone treatment, which blocks the antibodies from attacking the platelets. A splenectomy can increase the platelet count because the spleen can no longer attack the platelets. On the other hand, adults with mild ITP may not need to be treated at all.
ITP is treated with corticosteroids to interrupt the immune cycle. Some people receive intravenous IgG, which tricks the body by hiding the platelet receptors. Surgery is used to remove the spleen if no other treatment helps the problem. Immunosuppressant drugs, such as Cytoxan and Imuran, have many side effects but work to increase the platelet count.