This is the third part of our series about the condition based on our patient booklet “Fast Facts for Patients: Thrombotic Thrombocytopenic Purpura”. This article shows the different treatment modalities for thrombotic thrombocytopenic purpura (TTP), explains how your treatment is being monitored, and also focuses on the treatment of congenital TTP (iTTP) treatment.

Treatment of TTP

Both immune TTP (iTTP) and congenital (cTTP) are treated by replacing the missing ADAMTS13 enzyme. For iTTP, it is also essential that the immune system is suppressed to stop the production of antibodies that target ADAMTS13. Drug treatment can be given as well. Some patients may also need medicines to stop the body from making blood clots.

TTP treatment: plasma exchange for iTTP and plasma infusion for cTTP.

Monitoring Your Treatment

Because acute iTTP is life-threatening, it needs urgent intensive treatment. It is also very important that patients’ responses to treatment are monitored. This is done by monitoring symptoms and vital signs (like temperature, heart rate, breathing rate and blood pressure), and also blood markers such as platelet counts and ADAMTS13 enzyme levels.

Platelet Counts
  • These usually begin to recover from low levels back to normal a few days to 1–2 weeks after starting treatment.
ADAMTS13 Levels
  • These usually take a few weeks to return to normal.
  • It is important that your ADAMTS13 level keeps being monitored, even if you are well and have fully recovered from acute TTP (called remission).
  • If your ADAMTS13 level begins to drop, it could indicate that the antibody causing TTP is coming back and there is a risk of another acute TTP episode (called a relapse). This can be treated by giving a medication called rituximab electively (to clear the antibody and prevent a relapse) as an outpatient.

If a treatment is described as being elective it means that it is planned, as opposed to being given in an emergency.

Magnetic resonance imaging

Because TTP commonly affects the brain and the heart, you may have a brain scan called an MRI (magnetic resonance imaging) scan and a heart scan (an echocardiogram) to check that they are functioning normally.

It is very important to be followed up for TTP by a specialist.

Treatment for Congenital TTP (cTTP)

Treatment of cTTP focuses on replacing the missing ADAMTS13 enzyme.

Plasma Infusion

Infusions of plasma are given via a cannula inserted into a vein. The amount of plasma infused and the frequency of the infusions are based on symptoms (such as headaches) and blood test results, but infusions are normally 1–2 weeks apart. In some circumstances, recombinant ADAMTS13 enzyme may be given if you decide to take part in a clinical trial. See the next section for more information. During plasma infusion, patients with cTTP sometimes need to take medications that thin the blood, such as aspirin. Folic acid may also be taken to speed up the production of red blood cells. Response to plasma infusions in patients with cTTP is assessed by monitoring symptoms and blood test results. How much plasma you are given and how many infusions you have are based on this.

Plasma infusion for cTTP.

 

Please check out the other posts of our series here:

 

Information based on Fast Facts for Patients: Thrombotic Thrombocytopenic Purpura (Karger, 2022).

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