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Thrombotic Thrombocytopenic Purpura (TTP)

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Thrombotic Thrombocytopenic Purpura (TTP) is a rare disorder that causes clotting in the small blood vessels of many organs (microangiopathy) and results in organ damage, low platelets and anemia. Patients with this disorder may experience easy bruising, excessive bleeding, and anemia. If this occurs in the kidney there may be kidney failure. In the brain it can result in confusion or even coma.

Annually, there are four new cases of TTP per 100,000 people. It is most common in adults from 20-50 years old, and it affects women slightly more often than it affects men. There is no known cure.

Diagnostic Tests

To diagnose TTP, your doctor will order a blood test to determine blood counts, kidney function, and tests of red blood cell breakdown. If the results are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders.

Treatment Overview

Your treatment plan will be determined based on your age, overall health, if you are having any symptoms, and the results of your blood tests. Your treatment plan may include:

Plasmapheresis

In plasmapheresis, the blood is filtered through a machine that removes plasma and replaces it with plasma from healthy blood donors.

Infusion of New Plasma

In this treatment, plasma from healthy donors is given to slow down clotting in small vessels.

Corticosteroids

Corticosteroids are hormones produced naturally by the body to regulate the immune system. You may be given additional corticosteroids to help this regulation process. One type of drug often used for this is prednisone.

Other Treatments

Although rare, chemotherapy or splenectomy may be necessary if other therapies fail.

Managing Thrombotic Thrombocytopenic Purpura

In addition to the treatment recommended by your doctor, there are steps you can take to help reduce or prevent the symptoms of TTP:

  • Avoid bumping or bruising yourself.
  • Use an electric razor when shaving and be cautious when using nail trimmers, knives, etc.
  • Wear hard-soled shoes, gloves, and long pants when working outside (i.e. gardening).
  • Use a sponge toothbrush if you have problems with gum bleeding. Your doctor or nurse can tell you if you need to use one and where it can be acquired.
  • Avoid aspirin or aspirin-like medications (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless your doctor has told you otherwise. These medications can affect blood clotting. Also, be sure to inform your doctor of all over-the-counter medications that you take (including vitamins, herbs and dietary supplements).

It is important that you be alert for any change or increase in symptoms. If this occurs, it may mean that you need additional or different treatments, and you should contact your doctor right away. The following symptoms require prompt attention:

Bleeding Symptoms

  • unusually heavy or prolonged bleeding
  • bleeding for no apparent reason
  • frequent bruising
  • nose or gum bleeding
  • black, tarry, or bloody stools
  • blood in the urine
    • petechiae, or tiny areas of pinpoint bleeding on the skin of the arms and legs

Microangiopathic Clotting Symptoms

  • jaundice, yellowing of the skin or eyes
  • headaches
  • dizziness
  • confusion of speech or changes in speech
  • confusion

Anemia Symptoms

  • pale color
  • shortness of breath, especially during activity
  • weakness or fatigue

Other Symptoms

  • fever

Follow-Up Care and Prognosis

TTP requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment.

Prognosis depends upon the symptoms you may have, your age, and the treatment you receive. Some patients may only need minimal care, while others require more intensive treatment. If you have questions or concerns about your treatment and prognosis, do not hesitate to discuss these with your doctor. It may be helpful to write your questions out before your visit.