What is aTTP?

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening blood disorder. You may hear your doctor or some people refer to it as TTP. This is a general term. The 2 main types of TTP are inherited and acquired; however, 95% of all TTP cases are acquired.

One of the components in your blood that helps your platelets Platelets: A small cell found in the blood and involved in clotting. stick together is called von Willebrand factor (vWF) von Willebrand factor: One of the components in your blood that helps your platelets stick together. . Platelets are small cells found in the blood and are involved in clotting. Their main job is to form blood clots when bleeding happens. Platelets stick together to seal a cut or injury. This stops bleeding.

Normally, vWF is cut up in to smaller pieces by an enzyme called
ADAMTS13 ADAMTS13: An enzyme, or protein molecule, needed to prevent the body from making more blood clots than it needs. .

ADAMTS13 cuts vWF ADAMTS13 cuts vWF

In people with aTTP, the body’s immune system makes proteins called autoantibodies that stop ADAMTS13 from cutting up vWF.

ADAMTS13 does not cut vWF ADAMTS13 does not cut vWF

These large molecules of vWF attract platelets. This forms blood clots even when there is no bleeding. These clots:

  • Can block the flow of blood to important organs, such as the brain, heart, or kidneys. This can cause serious health problems, such as stroke or seizure
  • Can destroy red blood cells, leading to a condition called hemolytic
    anemia Anemia: A drop in the number of red blood cells in the body. Red blood cells carry oxygen throughout the body. . Because red blood cells carry oxygen, the body doesn’t get as much as it needs. This can make people feel tired and may cause a higher heart rate
Platelets stick together to form a clot Platelets stick together to form a clot

What causes aTTP?

It may not be known why someone develops aTTP, but the following could be triggers:

  • Certain conditions, including cancer, HIV, lupus, infections, or pregnancy
  • Surgery or certain other medical procedures
  • Certain medications, including chemotherapy, hormone therapy, and estrogen-based medicines (such as oral contraceptives)

Blood flow without aTTP

Normal blood flow

Blood clot risk in aTTP

Blocked blood flow

What are the signs and symptoms of aTTP?

Everyone’s experience with aTTP is different. You may have had some of the following symptoms. These can be signs of aTTP and could be a warning of having another aTTP episode. This is called a recurrence Recurrence: Any aTTP episode that follows the first aTTP event. .

Blood drop

Bleeding-related symptoms

  • Bleeding from the gums or nose
  • Purple bruises (known as purpura) and/or red or purple dots (known as
    petechiae Petechiae: Small red or purple spots less than 1/8 inch on the skin. ) on the skin
  • Blood in urine
  • Stomach pain
Blood clot

Blood clot–related symptoms

  • Chest pain
  • Seizures
  • Headaches, confusion, and distorted vision
  • Tiredness and jaundice (a yellowing of the skin and eyes)

IMPORTANT SAFETY INFORMATION

Who should not take CABLIVI?

Do not take CABLIVI if you’ve had an allergic reaction to caplacizumab-yhdp or to any of the ingredients in CABLIVI.

What should I tell my healthcare team before starting CABLIVI?

Tell your doctor if you have a medical condition including if you have a bleeding disorder. Tell your doctor about any medicines you take.

Talk to your doctor before scheduling any surgery, medical or dental procedure.

What are the possible side effects of CABLIVI?

CABLIVI can cause severe bleeding. In clinical studies, severe bleeding adverse reactions of nosebleed, bleeding from the gums, bleeding in the stomach or intestines, and bleeding from the uterus were each reported in 1% of subjects. Contact your doctor immediately if excessive bleeding or bruising occur.

You may have a higher risk of bleeding if you have a bleeding disorder (i.e. hemophilia) or if you take other medicines that increase your risk of bleeding such as anti-coagulants.

CABLIVI should be stopped for 7 days before surgery or any medical or dental procedure. Talk to your doctor before you stop taking CABLIVI.

The most common side effects include nosebleed, headache and bleeding gums.

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of CABLIVI. Call your doctor for medical advice about side effects.

INDICATION

What is CABLIVI?

CABLIVI (caplacizumab-yhdp) is a prescription medicine used for the treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy.

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