Thrombotic Thrombocytopenic Purpura - Symptoms, causes and treatment

Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that can make the blood clot more quickly. This disorder can cause blood flow to the body's organs to be blocked and can be fatal.

TTP is a rare disease with a potential incidence of only about 4 cases per 1 million people, and is more common in women. The main symptom of this disorder is the appearance of a purplish red rash due to bleeding under the skin. Symptoms can appear suddenly and last a few days to several months.

Causes of Thrombotic Thrombocytopenic Purpura

The exact cause of thrombotic thrombocytopenic purpura is not known. However, the disruption of the ADAMTS13 enzyme activity is thought to have contributed to the emergence of this disease. The ADAMTS13 enzyme is one of the proteins involved in the blood clotting process.

A deficiency of the ADAMTS13 enzyme can cause the blood clotting process to become very active, resulting in the formation of many blood clots throughout the body. As a result, the blood supply that carries oxygen to the body's organs, such as the brain or heart, becomes blocked.

The number of blood clots will make the number of platelet cells (platelets) decrease (thrombocytopenia). On the other hand, this drop in platelets will actually make the body more prone to bleeding.

Impaired function of the ADAMTS13 enzyme can be caused by inherited genetic disorders. Even so, the disorder is more often due to autoimmune diseases, in which the body produces other antibodies that destroy these enzymes.

In addition, TTP can also be triggered by the following conditions:

  • Certain diseases, such as bacterial infections, HIV/AIDS, inflammation of the pancreas, cancer, autoimmune diseases (eg lupus and rheumatoid arthritis), or pregnancy.
  • Medical procedures, such as organ transplant surgery, including bone marrow transplants.
  • Use of medications, such as ticlopidine, quinine, ciclosporin, clopidogrel, and hormone therapy.

Symptoms of Thrombotic Thrombocytopenic Purpura

Although there are genetic disorders that have been present since birth, generally the symptoms of thrombotic thrombocytopenic purpura only appear when the patient is an adult. Symptoms of TTP can appear from the age of 20 to 50 years. Thrombotic thrombocytopenic purpura is characterized by a number of skin symptoms, such as:

  • A red rash on the skin and mucous membranes, such as the inside of the mouth.
  • Bruises appear for no apparent reason.
  • The skin looks pale.
  • Yellowish skin (jaundice).

In addition to the above symptoms, TTP disease can also be accompanied by some of the following additional symptoms:

  • Fever
  • Body feels weak
  • Lost concentration
  • Headache
  • Decreased frequency of urination
  • Different heart
  • Hard to breathe

When to go to the doctor

Immediately consult a doctor if you experience the symptoms of thrombotic thrombocytopenic purpura mentioned above. Early treatment needs to be done to prevent serious complications.

TTP is a disease that can recur. If you have been diagnosed with this disease, do regular check-ups with your doctor to monitor the progress of the disease.

This disease can also be inherited genetically. Therefore, patients are advised to discuss their condition further with their doctor when planning to have children, so that this disease is not passed on to children.

People who are at risk of suffering from HIV/AIDS are more likely to develop TTP. Therefore, people with HIV/AIDS and people who are at risk of getting HIV/AIDS need to see a doctor to anticipate the emergence of TTP.

The same should be done by people who have recently undergone surgery or hormone therapy, and often take blood-thinning medications, such as ticlopidine and clopidigrel. Examination is needed to monitor the success of the action and anticipate possible side effects.

You need to go to the ER immediately if you experience symptoms of TTP accompanied by heavy bleeding, seizures, or symptoms of a stroke.

Diagnosis of Thrombotic Thrombocytopenic Purpura

The doctor will ask about the complaints and symptoms experienced by the patient as well as the medical procedures the patient has undergone. The doctor will also ask about the history of the patient and his family members.

Furthermore, a physical examination is performed primarily to assess the signs of bleeding and heart rate. If the patient is suspected of having TTP, a number of additional tests will be carried out to confirm it. The tests include:

blood test

The patient's blood sample will be tested in full, starting from the number of red blood cells, white blood cells, to platelets. Tests for bilirubin levels, antibodies, and the activity of the ADAMTS13 enzyme will also be performed on the blood test.

urine test

A urine test can be done to analyze the characteristics and amount of urine, and look for the presence or absence of blood cells or protein in the urine, which are commonly found in people with TTP.

Treatment of Thrombotic Thrombocytopenic Purpura

Treatment of thrombotic thrombocytopenic purpura aims to normalize the blood's clotting ability. Treatment needs to be done immediately, because if not, it can be fatal.

In general, TTP can be treated by the following methods:


Doctors can give a number of drugs to relieve symptoms and reduce the chance of recurrence of TTP. The drugs given include corticosteroids, vincristine, and rituximab.

Plasma exchange therapy (plasmapheresis)

Blood plasma exchange therapy can be used to treat TTP, because the ADAMTS13 enzyme which is suspected to be the cause of TTP is in the blood plasma.

In this therapy, the patient's blood will be drawn through an IV and transferred to a machine that can separate plasma from other parts of the blood. The patient's blood plasma is then discarded and replaced with healthy donor plasma.

The plasma exchange procedure usually lasts about 2 hours. Therapy needs to be done every day until the patient's condition really improves. During treatment, corticosteroid drugs can also be given by a doctor to increase the effectiveness of treatment.

Plasma transfusion

Plasma transfusion is common in TTP patients due to genetic disorders. TTP patients due to genetic disorders have a lack of plasma, so it is necessary to do blood plasma transfusions from donors.

Complications of Thrombotic Thrombocytopenic Purpura

If not treated immediately, thrombotic thrombocytopenic purpura can cause the following complications:

  • Kidney failure
  • Anemia
  • Nervous system disorders
  • Heavy bleeding
  • stroke
  • Infection

Prevention of Thrombotic Thrombocytopenic Purpura

Some patients make a full recovery from thrombotic thrombocytopenic purpura, while others may experience a relapse. There are no specific preventive measures to address this problem. What needs to be done is to reduce the risk of TTP recurrence, by avoiding triggers.

If you have a family who is or has ever experienced TTP, consult your doctor to find out if you have this disease as well. The reason, TTP can occur due to genetic influences.

If you have experienced symptoms of TTP, do regular health checks to a hematologist, even if you feel healthy. During each visit, don't forget to always tell your doctor about any medications you are taking, including vitamins and herbal remedies.