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Thrombotic thrombocytopenic purpura – Symptoms, diagnosis, and management

Thrombotic Thrombocytopenic Purpura, or TTP, is a rare but life-threatening blood disorder. In this condition, blood clots form in the small blood vessels throughout the body, which can limit the flow of blood and potentially block it from reaching the organs, such as the heart, kidney, and even the lungs. This means it can create serious complications and needs immediate treatment. Here are key things to know about the disease, its diagnosis, and the treatment options.

Symptoms and causes
TTP occurs when there is insufficient ADAMTS13 available in the blood. This protein is crucial in regulating how the blood clots. With the lack of this enzyme, excessive blood clotting can occur, leading to complications. This condition can be inherited from a parent’s gene or, in some cases, acquired by the person.

Some of the symptoms of TTP include petechiae, which are small, flat red spots on the skin caused by the leaking of blood in the blood vessels. Purpura is another symptom in which the bleeding causes red, purple, or brownish-yellow spots. Other signs include fast heart rate or shortness of breath, headache, confusion, coma, stroke, seizure, nausea, fatigue, fever, and diarrhea.

Diagnosis
The healthcare provider may ask for the patient’s health history and family history to check for any inherited diseases. Further, they may suggest some of the following tests to confirm the TTP diagnosis:

Blood smear: A blood sample is put on a slide and examined under a microscope to check for any torn or broken blood cells.

ADAMTS13 assay: Blood is drawn from a vein for this test, which is then sent to the lab to check the enzymes’ activity levels.

Bilirubin test: Because of TTP, the bilirubin levels may be high as the body tends to break down the red blood cells faster than its normal pace in this condition.

Coombs test: This is done to see if the condition is causing hemolytic anemia. In this condition, the red blood cells break into pieces because they try to squeeze around the blood clots. When the test is negative, it means that TTP is the cause of hemolytic anemia.

Some other tests to diagnose the condition involve kidney function tests, urine tests, lactate dehydrogenase tests, and a complete blood count test. All these help determine the condition better and propose a treatment plan accordingly.

Treatment options
Considering the severity of the condition, treatment must be administered quickly and immediately to manage it better. Here are some options:

Therapeutic plasma exchange – This type of treatment is done for acquired TTP. In this, the liquid part of the blood is replaced with the donor plasma, which helps remove the antibodies that damage the ADAMTS13 enzyme.

Plasma infusion – This is the method used to treat inherited TTP. The donor plasma is given through an IV or an intravenous line through the vein. This helps in replacing the missing or faulty enzyme.

Surgery – In severe cases, surgery may be necessary to treat the condition. The spleen is removed through a surgical procedure to help stop the making of the antibodies, which hinder the process of the ADAMTS13 enzyme. However, this treatment is a last resort if other treatments do not work.

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