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Idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura is the condition of having a low platelet count (thrombocytopenia) of no known cause (idiopathic). As most causes appear to be related to antibodies against platelets, it is also known as immune thrombocytopenic purpura. Although most cases are asymptomatic, very low platelet counts can lead to a bleeding diathesis and purpura. more...

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Signs and symptoms

ITP occurs most often in women over 40 years of age. It may be acute, lasting for 6 months or less, or chronic, lasting for over a year. The acute type is more often seen in children and will cure itself in more than 80% of cases. The chronic type is more commonly seen in adults and it tends to get worse as the disease progresses.

Occasionally, ITP patients suffer from bruising, nosebleeds, and bleeding gums; this is the characteristic pattern of bleeding in platelet disorders. Bleeding normally does not occur unless the platelet count is very low (below about 10,000 per mm3, compared to a normal range of 150,000–400,000 per mm3).

Subarachnoid and intracerebral hemorrhage are very serious possible complications of this disease. Fortunately, these are rare in patients who are being treated.

Pathogenesis

In many cases, the cause is not actually idiopathic but autoimmune, with antibodies against platelets being detected in approximately 80% of patients. Most often these antibodies are against platelet membrane glycoproteins IIb-IIIa or Ib-IX, and are of the IgG type. The coating of platelets with IgG renders them susceptible to opsonization and phagocytosis by splenic macrophages.

The IgG autoantibodies are also thought to damage megakaryocytes, the precursor cells to platelets, but this is thought to contribute only slightly to the decrease in platelet numbers.

Diagnosis

When measuring the platelet count, one has to bear in mind that the "normal values" for laboratory measures are all statistical. They are defined by the upper and lower 2.5th percentile. It is therefore possible to be completely healthy but to have a decreased platelet count. There is, however, a higher chance of pathology.

The diagnosis of ITP is a clinical one and is a diagnosis of exclusion. Low platelet count can be a feature of a large number of diseases and, when serious, warrants investigation by a hematologist. Secondary causes include leukemia, medications (e.g. quinine), lupus erythematosus and some other autoimmune disorders, cirrhosis (leading to thrombocytopenia from hypersplenism), HIV, congenital causes, and antiphospholipid syndrome. A bone marrow examination may be performed on patients over the age of 60 and people who do not respond to treatment, or when the diagnosis is in doubt.

Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged. In fact, an enlarged spleen should lead a clinician to investigate other possible causes for the thrombocytopenia.

Accelerated formation of platelets results in the presence of abnormally large platelets which are seen in a peripheral blood smear. Overall bleeding time is prolonged in these patients, but prothrombin time (PT) and partial thromboplastin time (PTT) are normal (because the problem is with the platelets, not with the coagulation cascade).

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The Official Patient's Sourcebook on Idiopathic Thrombocytopenic Purpura: A Directory for the Internet Age $32.71 Rituximab for immune cytopenia in adults: Idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome : An article from: Mayo Clinic Proceedings $20.00
Trigger autoimmunity--development of multiple plexopathy in a patient with chronic idiopathic thrombocytopenic purpura.(Letter to Editor)(Letter to the Editor) : An article from: Neurology India $5.95 Inclusion body myositis associated with celiac sprue and idiopathic thrombocytopenic purpura. (Case Report). : An article from: Southern Medical Journal $5.95
The Official Patient's Sourcebook on Idiopathic Thrombocytopenic Purpura: A Revised and Updated Directory for the Internet Age $28.95 Idiopathic Thrombocytopenic Purpura - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References $34.95
21st Century Complete Medical Guide to Bleeding Disorders, Thrombocytopenia, von Willebrand Disease (VWD), Idiopathic Thrombocytopenic Purpura (ITP), Authoritative ... Information for Patients and Physicians $16.50 Gale Encyclopedia of Medicine : Idiopathic thrombocytopenic purpura $5.50
Diagnosis and treatment of idiopathic thrombocytopenic purpura : An article from: Journal of the American Academy of Nurse Practitioners $20.00 Accessory spleens in the thoracic and abdominal cavities after a relapse of idiopathic thrombocytopenic purpura: A case report : An article from: Journal of Nuclear Medicine Technology $20.00

Idiopathic thrombocytopenic purpura
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