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TTP or Thrombotic Thrtombocytopenic Purpura

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  • Remicade and TTP (Thrombotic Thrombocytopenic Purpura)

Thrombotic thrombocytopenic purpura, or TTP is a rare blood disorder that causes clots to form in the body’s small blood vessels. While only diagnosed at a rate of 3-4 in 1 million people per year, TTP can cause severe damage to critical organs, including the brain, heart and kidney, as well as other life-threatening health complications.

What Causes TTP?

It’s not clear what causes TTP. For some reason, the blood becomes “sticky,” causing platelets to clot in the small blood vessels throughout the body. These clots impede blood flow to vital organs such as the brain, heart and kidneys.

Because they are being used by the body to form numerous unnecessary clots, platelets are unable to perform their normal function: sealing wounds to prevent excessive bleeding due to an injury. As such, patients with TTP are at risk for life-threatening bleeding when they suffer otherwise minor traumas.

Patients who are born with TTP are often deficient in an enzyme called ADAMTS 13, which plays a role in blood-clotting. However, this deficiency is not always seen with adult-onset TTP.

Types of TTP

There are several types of TTP:

• Hereditary TTP: Caused by an inherited deficiency or abnormality of the ADAMTS 13 enzyme. This type accounts for less than 10% of all TTP cases. Other names for inherited TTP include familial TTP and Upshaw-Schulman syndrome (USS).
• Idiopathic or Acquired TTP: 45% of all TTP cases have no defined cause. These patients may have decreased levels of the ADAMTS 13 enzyme. Alternative names for idiopathic/acquired TTP include Moschcowitz disease and microangiopathic hemolytic anemia.
• Secondary TTP: 45% of TTP cases are secondary to a pre-disposing factor, such as: autoimmune diseases, cancer, bone marrow transplantation, pregnancy, HIV infection, pancreatitis and hepatitis. Certain medications can also cause secondary TTP. For example, the prescribing information for Remicade lists TTP as an adverse event that has been reported among patients since the drug’s approval. Generally, patients with secondary TTP do not suffer from ADAMTS 13 enzyme deficiency.

TTP Symptoms

The signs and symptoms of TTP include:

• Purpura: Purplish bruises on the skin or mucous membranes that are caused by bleeding under the skin.
• Petechia: Pinpoint-sized red or purple dots on the skin. Petechia are often found in groups and can resemble a rash. They are also caused by bleeding under the skin.
• Paleness
• Bleeding (from nose and/or gums)
• Jaundice (yellowing of the skin or eyes)
• Fatigue
• Fever
• Rapid heart rate
• Shortness of breath
• Chest pain
• Neurologic symptoms (headache, changes in speech, confusion, etc.)
• Decrease in amount of urine
• Protein or blood in the urine
• Kidney failure

Treatment of TTP

Without treatment, TTP can result in lasting damage or even death.

TTP is treated with plasma therapy in the form of either fresh frozen plasma or plasma exchange.

Hereditary TTP is treated with fresh frozen plasma to replenish the ADAMTS 13 enzyme. The plasma is administered through an intravenous (IV) line inserted into a vein.

Acquired or secondary adult TTP is treated via plasma exchange (plasmapheresis). This process removes antibodies from the blood that damage the ADAMTS 13 enzyme. During the procedure, an IV needle or tube is placed in the patient’s vein to remove blood. The blood then goes through a process to separate the neoplasm from the plasma. Healthy, donated plasma is then added to the patient’s neoplasm. Finally, the reconstituted blood is returned to the body through an IV.

Glucocorticoids, vincristine, rituximab, and cyclosporine are used to treat acquired TTP if plasma therapy doesn’t work or if a patient experiences frequent recurrence.

Sometimes surgery to remove the spleen – which produces antibodies that inhibit the ADAMTS 13 enzyme – is needed.

TTP Prognosis

Many TTP patients fully recover with appropriate treatment. However, relapses (flareups) can occur in many people who have acquired and inherited TTP. Early detection of a flareup is vital to reduce the risk of irreversible injury or death.