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What is Idiopathic Thrombocytopenic Purpura?

written by: Blaise Wellesley • edited by: Leigh A. Zaykoski • updated: 9/30/2010

Find out exactly what idiopathic thrombocytopenic purpura is, how to treat it, and how aging influences the disorder.

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    What Is Idiopathic Thrombocytopenic Purpura?

    Idiopathic thrombocytopenic purpura, or ITP, is a blood disorder in which the blood does not clot as well as it should. This occurs from a decreased number of blood cell fragments called platelets. Platelets are used through your blood stream to clot together to seal small ruptures in walls to stop bleeding. Platelets are also called thrombocytes.

    When a patient has idiopathic thrombocytopenic purpura, they usually display purple bruises on their skin, signifying bleeding under the skin. The word idiopathic means that the cause of onset of itp blood disorder is not yet known. So with all the words together, Idiopathic thrombocytopenic purpura means, a disease with a low number of blood platelets which increases bruising under the skin, for which the cause is not yet known.

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    Origin

    Idiopathic thrombocytopenic purpura was found and noted by Paul Werlhof, a German physician in 1735, and it was named Werlhof’s Disease at the time. Until the introduction of steroid therapies in response to idiopathic thrombocytopenic purpura, a splenectomy was seen to cure the blood disorder. A splenectomy is a removal of the spleen. As the spleen is a large lymph node, it filters out damage blood cells, including platelets. The spleen can engorge and eventually rupture causing death. With the spleen gone, the blood system can focus on making successful platelets, albeit decreased levels, but effective.

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    Diagnosis and Prevalence

    An average blood platelet count in an individual not affected with idiopathic thrombocytopenic purpura is around 150,000 to 450,000 per cubic millimeter. A patient is considered to be thrombocytopenic when their levels fall below this range. As there no set numbers to diagnose idiopathic thrombocytopenic purpura, many doctors rely on their intuition and other tests to make the diagnosis.

    Roughly 50 to 100 new cases per million happen per year, usually with children accounting for more than half of the incident rate. However, approximately 70 percent of infant and childhood cases will end up in remission without the use or need of treatment. A third of all chronic cases will pass on during an observation, and another third will have a slightly decreased, but less harmful, platelet count.

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    Issues with Aging

    Infection through adults is usually chronic, with the likelihood of remission being around 30 to 40 percent. The average age of diagnosis in adults is in the later 50s to the early 60s. In terms of gender, the male to female ratio is around 1:1.2 to 1.7 in most age spans. However, childhood cases are generally equal in comparision to age range, whereas the ratio begins to widen with age. In the United States only, the inflicted adult total population is around 60,000 and the ratio of genders is 2:1 with women outnumbering men. Because of these statistics, ITP blood disorder has since been declared an orphan disease. An orphan disease is considered when the total number of infected patients is lower than 200,000, making it financial ineffective for pharmaceutical companies to produce new medications.

    As the mortality rate of chronic idiopathic thrombocytopenic purpura varies, it increases directly with age. With a study concluded in the United Kingdom, patients with idiopathic thrombocytopenic purpura experienced roughly 60 percent higher rate of mortality than that of their equally comparable gender and age matched peers without the diagnosis of idiopathic thrombocytopenic purpura. The threat of increases risk in mortality occurs mainly in middle and elderly aged patients. Approximately 96 percent of reported deaths were patients 45 years of age or older. There was, however, no scientifically significant different between the rates of survival and death in males and females.

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    References

    • http://www.nhlbi.nih.gov/health/dci/Diseases/Itp/ITP_WhatIs.html
    • http://www.mayoclinic.com/health/idiopathic-thrombocytopenic-purpura/DS00844
    • http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/hematology/bledidio.html