What It Means

Thrombotic- Clotting in the Blood.

Thrombocytopenic- Reduction in the number of platelets in the blood.

Purpura- Bleeding in the skin causing purple spots/rash.

What is TTP

In TTP platelets clump together and form small clots, typically in the blood vessels supplying the brain and kidneys.As a result, platelets are consumed and the number of platelets circulating in the bloodstream falls dramatically.The clots may damage red cells as they pass through. The damaged cells are subsequently removed from the circulation and anaemia may result.This is NOT the same as ITP (Idiopathic Thrombocytopenic Purpura) when platelets are preferentially cleared from the bloodstream as they are covered by antibodies directed against platelets. For more information on itp please click here for the support association.

 What Happens

Small platelet clots form and patients may develop renal impairment or neurological problems such as headache, poor memory, mini-strokes or fits. Another common symptom is fever. Less commonly some also experience adbominal pain or visual problems. Although bruising is frequent, overt bleeding is rare.

Who Gets It

TTP affects approximately one in one million people per year .It may occur at any age although those most commonly affected are 30-40 years old. Similarly although both men and women may develop TTP, women are more often affected.

Why Does It Develop

Until recently the precise cause of TTP has been a mystery. However recent research suggests that the processing of von Willebrand factor, a protein normally found in blood, is impaired and as a result abnormally Iarge forms of the protein circulate.

Although not yet isolated it is likely that an enzyme is normally responsible for this. Some people may be deficient in this enzyme and in others it may not be able to work efficiently due to an inhibitor. Therefore TTP may have different causes in different people.

How is It Treated

Treatment can be by way of Plasma Exchange or Plasma Transfusion.Plasma Exchange is to remove the Plasma element from the patients whole blood by a centrifuge or filter within a machine. The patients own blood is then replaced together with the addition of fresh frozen doner plasma. (FFP)Some patients may require steroids and or chemotherapy which acts to suppress the immune system.