What is thrombotic thrombocytopenic purpura (TTP)?

TTP is a blood disorder that causes anemia and bleeding problems due to low platelet levels. Anemia is low levels of red blood cells. Without enough platelets, you can bleed in your organs, underneath your skin, or from the surface of your skin. TTP can also cause problems with kidney and brain function.

What causes TTP?

TTP is caused by the lack of a blood protein that controls how platelets work. Any of the following may cause this to happen:

• Certain genes that are passed from parents to children
• Medical conditions such as cancer, HIV, lupus, or infections
• Pregnancy
• A medical procedure such as a bone marrow transplant
• Medicines such as chemotherapy, blood thinner medicine, and hormone therapy
• Exposure to quinine, a substance found in tonic water and other nutrition products

What are the signs and symptoms of TTP?

• Red or purple dots or bruises on the skin or mucus membranes (such as in the mouth)
• Paleness or jaundice (yellow coloring of the skin or whites of the eyes)
• Fatigue, achiness, weakness, or fever
• Headache, confusion, or being less awake than usual
• Urinating less than usual or not at all
• Abdominal pain, nausea, or vomiting

How is TTP diagnosed and treated?

Your healthcare provider will examine you and ask about your symptoms. You will need blood and urine tests to measure your platelet levels and kidney function. Medicines that can cause TTP may be stopped or changed. You may also need any of the following:

• A plasma transfusion can help improve your platelet levels and control how your blood clots. Plasma is the liquid that surrounds your blood cells.
• Plasma exchange is a procedure that removes antibodies from the blood. This procedure can help improve your platelet levels and control how your blood clots.
• Surgery to remove your spleen may be needed if your TTP is severe.

What can I do to care for myself?

• Do not take over-the-counter medicines before you talk to your healthcare provider. This includes NSAIDs, vitamins, and herbal supplements. These medicines can make TTP worse. Ask your healthcare provider for a full list of medicines you should not take.
• Do not drink or eat anything that contains quinine. Quinine can make TTP worse. Quinine is found in tonic water, flavored drinks, and some foods. Read all food and drink labels to check for quinine. Ask your healthcare provider for a full list of drinks and foods that contain quinine.
• Do not play contact sports. Examples of contact sports include football, wrestling, boxing, or hockey. Ask your healthcare provider which activities are safe for you.

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

• Cablivi
• caplacizumab

Call 911 for any of the following:

• You have any of the following signs of a heart attack:
  • Squeezing, pressure, or pain in your chest
  • You may also have any of the following:
    • Discomfort or pain in your back, neck, jaw, stomach, or arm
    • Shortness of breath
    • Nausea or vomiting
    • Lightheadedness or a sudden cold sweat
• You have any of the following signs of a stroke:
  • Numbness or drooping on one side of your face
  • Weakness in an arm or leg
  • Confusion or difficulty speaking
  • Dizziness, a severe headache, or vision loss
• You cough up blood.
• You have trouble breathing.

When should I seek immediate care?

• Your heart is beating faster than usual.
• You urinate less than usual or not at all.
• Your skin or the whites of your eyes are yellow.
• Your arm or leg feels warm, tender, and painful. It may look swollen and red.

When should I contact my healthcare provider?

• You have a fever.
• You have more bruises or small red or purple dots on your skin or mucus membranes.
• You have questions or concerns about your condition or care.

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