Idiopathic thrombocytopenic purpura
Idiopathic Thrombocytopenic Purpura (ITP) is an autoimmune disorder caused by the production of defective antibodies. Normally, antibodies are used by the immune system only to fight bacteria, viruses, or other harmful organisms. With ITP, however, antibodies also attack the platelets, causing their premature death and a decline in their overall production. The result of this is easy bruising or bleeding with no apparent injury.
Every year, there is one new case of ITP per 100,000 people. It most often develops in women between the ages of 20 and 40, but it can occur at any age. There is no known cure.
To diagnose ITP, your doctor will order a blood test to determine the number of platelets in your blood. If the results are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders. The hematologist may then test you further by doing a bone marrow biopsy. This consists of removing a small amount of bone marrow and examining it for abnormalities.
The primary goal of treatment is to slow the destruction and increase the number of platelets in the blood. The treatment you receive will depend on your age, health, if you are having any symptoms, and the results of your blood test and biopsy. It may include:
- Intravenous Immune Globulin
- Win Rho Immune Globulin IV
- Protein A Column Therapy
- Rituximab (Rituxan)
In some patients, the best treatment is to monitor the disorder for any increase in symptoms and have a doctor do periodic blood counts. This may be the only treatment ever needed.
Corticosteroids are hormones produced naturally by the body to regulate the immune system. You may be given additional corticosteroids to slow down the destruction of platelets and decrease the production of inappropriate antibodies. The drug often used for this is prednisone.
Chemotherapy uses medications to regulate the production of certain cells. In ITP, chemotherapy is used to slow the formation of cells producing inappropriate antibodies in the immune system. Some types of medications used include azathioprine, vincristine, cyclophosphamide, cyclosporine, or vinblastine.
Androgens are natural hormones that are occasionally helpful in ITP. One androgen used is Danazol.
Plasmapheresis can be used to temporarily reduce the amount of antibodies in the blood. During this procedure, blood is filtered through a machine that removes the antibody-containing plasma and replaces it with a substitute.
Intravenous immune globulin
Intravenous Immune Globulin is a special globulin with many antibodies that is given into the veins. Its therapeutic effect is temporary and there can be side effects during infusion.
Win rho immune globulin IV
Win Rho Immune Globulin IV is a special antibody that can slow platelet destruction. It can be used if the patient is Rh positive and has not had a splenectomy. Its effect is often temporary.
Protein a column therapy
A therapy in which the patient's plasma is passed through a special column to remove the antibodies which cause the platelets to be destroyed.
Ribuximab is an antineoplastic agent known as a monoclonal antibody. In ITP, it is used to decrease the number of cells producing inappropriate antibodies.
Managing idiopathic thrombocytopenic purpura
In addition to the treatment recommended by your doctor, there are steps you can take to help reduce or prevent the symptoms of ITP:
- Avoid bruising or bumping yourself, especially your head.
- Use an electric razor, and be cautious when using nail trimmers, knives, etc.
- Blow your nose very gently and maintain adequate room moisture to prevent nosebleeds.
- Wear hard-soled shoes, gloves, and long pants when working outside (i.e. gardening).
- Use a sponge toothbrush if you have problems with gum bleeding. Your doctor or nurse can tell you if you need to use one and where it can be acquired.
- Avoid aspirin or aspirin-like medications (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless your doctor has told you otherwise. These medications can affect blood clotting. Also, be sure to inform your doctor of all over-the-counter medications that you take (including vitamins, herbs and dietary supplements).
It is important that you be alert for any change or increase in symptoms. If this occurs, it may mean that you need additional or different treatments, and you should contact your doctor right away. The following symptoms require prompt attention:
- easy bruising
- bleeding for no apparent reason
- unusually heavy or prolonged bleeding
- petechiae, or tiny areas of pinpoint bleeding on the skin of the arms or legs
- blood in the urine or stools
- bleeding from the nose or gums
- severe headache or visual changes
- stiff neck
Follow-up care and prognosis
ITP requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment.
Prognosis depends upon the symptoms you have, your age, and the treatment you receive. Some patients need minimal care and observation, while others require more intensive treatment. If you have questions or concerns about your treatment and prognosis, do not hesitate to discuss these with your doctor. It may be helpful to write your questions out before your visit.