Thrombotic Thrombocytopenic Purpura (TTP) is a rare sort of blood disorder where blood clots are formed throughout the body in small blood vessels. The clots can restrict the flow of oxygen-rich blood to different organs of the body like the heart, kidneys, and the brain, and thereby lead to serious diseases and health ailment. This condition is caused due to lack of functioning in the ADAMTS13 enzyme. This enzyme is typically a protein found in the blood, and is responsible for blood clotting. Lack of enzyme activity leads to hyperactive blood clotting. People who are diagnosed with TTP usually show symptoms of internal bleeding, or abnormal bleeding when injured or cut.
Your doctor will perform a diagnosis depending on a physical examination, medical history, and test results. The following tests are usually conducted to confirm the diagnosis. Complete blood count- A blood sample is drawn from the veins in your arm to measure the count of platelets, red blood cells and white blood cells. People suffering from TTP have lower number of red blood cells and platelets than the normal measure. Blood smear- A blood sample is drawn from the veins in your arm, and the sample is put under a microscope to examine the red blood cells. The red blood cells appear broken and torn in TTP. Bilirubin test- A blood sample is drawn from the veins in your arm and is checked for bilirubin. People who have TTP have higher level of bilirubin since their body breaks down red blood cells quicker than normal. Kidney and Urine tests- These tests are performed to examine if your urine contains blood cells or protein. If you have TTP, the level of blood creatinine may be high in your body. Coombs test- This test determines TTP is caused due to hemolytic anemia.
Treatment of TTP is a plasma therapy.
The most common and effective treatment for TTP is a plasma therapy. Plasma is the liquid present in your blood which carries hormones, blood cells, nutrients and enzymes to the body. The treatment should start as soon as the diagnosis is confirmed. If you are diagnosed with Inherited TTP, frozen fresh plasma will be inserted through an intravenous line into your veins. This is done to substitute the changed or missing ADAMTS13 enzyme. For people with Acquired TTP, plasma exchange or Plasmapheresis will be performed. This is a lifesaving treatment procedure which removes proteins or antibodies from the blood which destroy the ADAMTS13 enzyme. It can also replace the ADAMTS13 enzyme. While performing plasma exchange, an intravenous tube or needle is placed into the vein to remove blood. The blood runs through a cell separator that separates plasma from the blood. The part of the blood which has no plasma is then saved, and the donated plasma is added to it. Finally, the blood is put back in your body through the intravenous tube injected in your blood vessel.
If plasma therapy fails, or if you develop flare ups during the treatment, your doctor will prescribe you certain medications such as rituximab, glucocorticoids, cyclosporine A, and vincristine. In other cases, you may have to get a surgery to remove your spleen, because the cells present in the spleen create antibodies which block the activity of ADAMTS13 enzyme.
People showing symptoms of internal bleeding or abnormal bleeding when injured or cut most likely suffer from Thrombotic Thrombocytopenic Purpura (TTP), and hence are eligible for the treatment. You may also observe signs of bruises on your skin, purple spots on the skin, fatigue etc.
If you do not show symptoms of TTP then you are not eligible for the treatment.
There are no known side effects of TTP treatment. However, people who have had their spleen removed, may develop infections or high fever. Oral drugs like glucocorticoids can adversely affect your health and increase the chances of diabetes, gastritis or ulcer, osteoporosis and hypertension.
Post treatment guidelines primarily involve following the prescribed medication, avoiding strenuous physical activity, and maintaining a proper diet to regulate the blood flow. Avoid using any product that contains quinine, as it may trigger TTP or a relapse of the condition.
It usually takes a few weeks to recover from TTP. But in some cases, the condition can last for a few months. In most cases, flare up or relapse also occurs even after the treatment. In order to avoid relapse of TTP make sure you follow the post treatment guidelines.
In India the price of one cycle of IVIg or four injections of rituximab amounts to Rs.1.2 lakh or more, while 5-6 sessions of plasma exchange amounts to around Rs.60,000 or slightly more.
The treatment results of TTP are mostly long lasting but not permanent as the condition may relapse or lead to a flare up after a certain period, depending on your health condition.
If plasma therapy fails, or if you develop flare ups during the treatment, your doctor will prescribe you alternative medications such as rituximab, glucocorticoids, cyclosporine A, and vincristine.