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Madisons Foundation - Moms And Dads In Search Of Needed Support
Idiopathic Thrombocytopenic Purpura
Monday, 18 October 2004
Last Updated Monday, 29 November 2004
What
Idiopathic Thrombocytopenic Purpura (ITP) is the development of purpura, which are purplish areas on the skin where superficial bleeding has occurred, due to low platelet levels (thrombocytopenia). It is an autoimmune process, where the body’s immune system attacks and destroys platelets necessary for blood clotting, resulting in bleeding and easy bruising. The cause of ITP is unknown or idiopathic.Who
ITP occurs in one of 20,000 children born per year. Girls are affected two to three times more frequently than boys. It most commonly affects children 2-4 years old. In children less than 10 years, ITP usually lasts less than 6 months and is not associated with other underlying illnesses. Adolescent females may develop a more chronic form of ITP, where recovery is prolonged.Signs and Symptoms
Acute ITP develops abruptly in an otherwise healthy child. Symptoms include gum/nose bleeding, easy bruising, purpura and petechiae (tiny red spots on the skin usually on the lower legs or inside the mouth resembling a rash). Fever and fatigue are uncommon and the child otherwise feels well. If platelet counts are very low, bleeding may occur in the stool and/or urine. The most worrisome location for bleeding is in the brain. If this occurs, the child may appear sleepy and confused or complain of a severe headache. Bleeding in the brain is very dangerous and needs immediate medical attention. Unlike children with hemophilia, ITP patients are not at risk for deep joint/muscle bleeding. Enlargement of the spleen, liver, and lymph nodes are also unusual in ITP and raise the concern for leukemia. Chronic ITP presents more gradually. Female adolescents with ITP may present with menorrhagia (very heavy menstrual periods).Possible Causes
ITP is not hereditary, contagious or preventable. It occurs more commonly in late winter and spring. Typically, children who develop ITP have a history of an upper respiratory infection 2-3 wks prior to the episode. Viruses such as Cytomegalovirus, Epstein-Barr virus, Hepatitis viruses, and HIV are known to trigger an episode of ITP. The MMR vaccine may also be associated with ITP. The body naturally makes antibodies to help identify the virus for destruction, and these antibodies then mistakenly cross-react with proteins found on platelets. The antibody-coated platelets are then destroyed by the spleen. One third of children with chronic ITP have other autoimmune disorders, such as lupus. In these children, there is an inherent dysfunction of the immune system.Diagnosis
The diagnosis of ITP is usually suggested by the child’s history and physical examination. Blood tests show a low platelet count. Under the microscope, giant platelets are seen because the bone marrow is working extra hard to produce more platelets. Anemia, or low hemoglobin, may be present if significant bleeding has occurred. cancer cells are absent in the blood. If the diagnosis is not clear or response to treatment is poor, a bone marrow biopsy can be done to confirm the diagnosis of ITP and exclude leukemia or bone marrow failure. This biopsy procedure involves inserting a needle into the pelvis bone to sample the marrow where blood cells and platelets are made. The area is anesthetized to minimize pain prior to the procedure and younger children may need sedation. In ITP, the bone marrow biopsy will be normal. In children with chronic ITP, additional blood tests to exclude thyroid disease and lupus may be performed.Treatment
Children with platelet counts below 20,000 are at increased risk for spontaneous bleeding and should be treated or followed very closely. Your doctor will likely consult a hematologist, a blood doctor. The most commonly used therapy is intravenous infusion of immuno globulin (IVIG, antibodies pooled from blood donors). These antibodies bind to the antibodies in the body that are attacking the platelets and stop platelet destruction. Platelet counts usually begin to recover within 3 days of IVIG therapy. Oral steroids, such as prednisone, can be used as well; however, a bone marrow biopsy to exclude leukemia is a prerequisite, as steroid therapy can mask underlying cancer. RhIG (RhoGAM) is another medication used in ITP. It is an immunoglobulin, or antibody, directed against the Rh protein on red blood cells. It is commonly used in pregnant women who are Rh negative to prevent red blood cell (RBC) destruction in the Rh positive baby. In ITP however, Rh positive patients are given RhIG to coat their RBCs with antibody. The spleen, then, becomes occupied with destroying the red blood cells rather than the platelets. Anemia can occur as a side effect. If the patient does not respond to the above medications and the course becomes chronic, immunosuppressive medications may be used. Platelet counts must be monitored regularly. It may take several years for recovery to occur. Surgery to remove the spleen results in long-term remission in some children with chronic ITP who have very low platelet counts and failed to respond to all other medications. The child with ITP may need blood transfusion if bleeding is severe. Platelet transfusion is not routinely performed because the body will destroy the platelets very quickly because it is making antibodies against the platelets. platelets are given only during very severe bleeding such as in the brain.Prognosis
Spontaneous recovery occurs in 80% of children with ITP. Younger children recover more quickly than adolescents. Older age increases the risk of severe bleeding. Ten to twenty percent of children may go on to have chronic ITP. Half of these children will eventually recover, while the rest may have ITP for life. Death occurs in 1% of children with ITP due to hemorrhage. Until platelet counts are completely recovered, aspirin and other blood thinning medications must be avoided to prevent bleeding. The child must refrain from contact sports and must wear protective gear, such as helmets, during physical activity.Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
The Platelet Disorder Support Association
A non-profit corporation providing information and support about ITP
The ITP Support Association
UK registered charity providing helpline support, a telephone contact and e-mail network with other parents, and a newsletter.