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Thrombotic Thrombocytopenic Purpura

Updated on February 17, 2014

Thrombotic thrombocytopenic purpura is a blood disorder which involves the formation of blood clots all over the body. This very rare disease is typified by clotting of the blood within the small blood vessels. This could result in low platelet count since the numerous small blood clots use up the platelets in the blood. As a consequence, the body may not be able to prevent bleeding when it gets injured because of the blood's inability to form clots. In addition, the clots may cause blockage within the blood vessels, hence preventing the proper circulation of blood throughout the body and hinder the organs from functioning correctly. When thrombotic thrombocytopenic purpura is in its advanced stage it will manifest various medical conditions, namely, renal problems, microangiopathic hemolytic anemia, neurologic abnormalities, thrombocytopenic purpura and fever.

What causes thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura or TTP could either be genetic or acquired. Genetic TTP is an autosomal recessive trait, which means both parents have the defective ADAMTS13 gene but do not necessarily display the symptoms. A problematic ADAMTS13 gene hampers the production of an enzyme which allows normal blood clotting. This type of TTP could show itself early in life or in adulthood.

On the other hand, acquired TTP occurs when the body manufactures proteins that prevent the enzyme from performing its job. The acquired type could arise from different conditions and usually develops in the later part of life. Adult-onset TTP affects those aged between 20 and 40 years old. It is extremely rare as it is estimated to affect only one in 3 million annually.

Factors that may bring forth TTP include:

  • compromised immune system (such as in the case of HIV)

  • cancer

  • infections

  • Systemic lupus erythematosus

  • pregnancy (specifically in the second trimester)

  • medical procedures like surgery and bone marrow transplant

  • use of drugs, including cyclosporine A, estrogen, chemotherapy and oral contraceptive pills

What are the symptoms of thrombotic thrombocytopenic purpura?

TTP first presents itself with mild and inconspicuous signs such as headache, body malaise, fever and diarrhea. Then, when small blood clots or thrombi begin to form in the blood vessels, bruises or purpura will become noticeable. These bruises are purple in color, although, small purple or red spots that resemble a rash may also develop. Bleeding in the gums and nose may occur, although rarely. The body could likewise develop jaundice, or a yellowish coloring of the skin.

The tiny blood clots may result in abnormal blood circulation, especially in those vessels that supply the kidneys and the brain. As a result, an individual affected by TTP will suffer from headache, dizziness, confusion, seizures, numbness, speech difficulties, anemia, change in mental status, visual disturbance and transient paralysis. For very severe cases, TTP may cause serious internal hemorrhage and stroke. Furthermore, cases where comma ensued had been recorded.

How is thrombotic thrombocytopenic purpura diagnosed?

To confirm the presence of TTP, a series of tests and diagnostic procedures may be required. A physical examination will be done to check the physical signs like bruising and spots on the skin. Blood work, such as blood film, complete blood count, platelet count, coagulation studies, blood smears and others will be performed to determine the condition. The blood will be examined for the low level of platelets and high bilirubin, creatinine, antibodies, lactate dehydrogenase and ADAMTS13 enzyme activity.

How is thrombotic thrombocytopenic purpura treated?

Treatment of TTP primarily aims to return the clotting ability of the blood to normal. This could be accomplished through plasma exchange, use of certain medications and even surgery.

  • Plasma exchange

Plasma is the part of the blood that has the vital clotting components. In plasma exchange, the patient's plasma in taken out from the blood and replaced with donor plasma. The objective of this is to eliminate the antibodies that attack the ADAMTS13 so as to allow it to function properly. Aside from using a plasma donor, the use of frozen plasma is an alternative. In fact, this is the treatment of choice for inherited TTP. The frozen plasma is made up of sterile human plasma from various human donors, which is treated to reduce the chances of infection. Plasma exchange is performed daily for a number of days until the condition gets better.

  • Drugs

Medications are prescribed in combination with the plasma exchange procedure. These may include steroids (corticosteroids), folic acid supplements and aspirin. In cases where plasma exchange fails, immunosuppresants to prevent the body from attacking the ADAMTS13 enzyme will be prescribed. Some of these immunosuppressive drugs are Ciclosporin, Cyclophosphamide and Azathioprine.

  • Surgery

Removal of the spleen may be necessary in some cases of TTP.

  • Transfusion

Transfusion of Red blood cells is needed for patients who develop anemia. Meanwhile, for those who suffer from low platelet count and uncontrollable bleeding, blood platelet transfusion can be done.

Thrombotic thrombocytopenic purpura is a grave condition. But with plasma exchange, the chance of survival is now estimated at 80%. Still, TTP may recur so it is recommended for patients to be monitored and immediately seek medical attention if symptoms reappear.


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