Printer friendly PDF versions in English and professionally translated French, Italian, Portuguese, Punjabi, Russian and Simplified Chinese can be found HERE. Please note that German and Spanish versions are also available HERE, but those translations have been generously completed by volunteers and the accuracy of the content has not been validated. The content is intended for informational purposes only, and is not meant to substitute consultation from a recognized health professional.
What is TTP?
Thrombotic Thrombocytopenic Purpura is a rare autoimmune blood disorder that is considered a true medical emergency. TTP is diagnosed at a rate of 3-4 in 1 million people per year. Potentially fatal complications can result from internal blood clotting with damage to critical organs such as the brain, heart and kidneys.
The cause of TTP continues to evade us. What is known is that blood becomes "sticky" and forms clots in blood vessels throughout the body. These clots are made up of platelets, one of the elements in blood. Vital blood flow to the body's organs is restricted, placing the organs at risk for damage due to a lack of oxygen and nutrients from the blood.
Moreover, since platelets are being used to form numerous unnecessary blood clots, their availability to perform their normal function, which is to seal injury sites to prevent excess bleeding, is compromised. Therefore life threatening bleeding may occur.
Research has shown that in some cases the ADAMTS 13 enzyme is deficient. This finding can be used to explain blood clotting; however, while ADAMTS 13 enzyme deficiency is found in congenital TTP cases, this is not always true of adult acquired cases of adult TTP. So we know that there is more to the recipe for TTP. Much more research is required!
Types of TTP
Hereditary TTP: Less than 10% of TTP cases are due to an inherited deficiency or abnormality of the ADAMTS 13 enzyme.
Idiopathic or Acquired TTP: 45% of TTP cases are of the idiopathic form, meaning there is no defined cause. Some cases have been linked to a decreased level of the ADAMTS 13 enzyme as a result of antibodies to the enzyme.
Secondary TTP: 45% of TTP cases are of the secondary form which is diagnosed when a predisposing factor is present including: autoimmune diseases, cancer, bone marrow transplantation, pregnancy, use of certain medications (quinine, platelet aggregation inhibitors, and immunosuppressants), HIV infection, pancreatitis and hepatitis. Usually ADAMTS 13 activity is normal in secondary TTP.
- Bleeding (from nose, gums)
- Chest pain
- Kidney failure (dark urine, jaundice)
- Neurologic Symptoms (confusion, headaches, visual changes)
- Thrombocytopenia (bruising, purpura, petechiae)
There is still much to learn about the mechanism that causes TTP. But it has been generally accepted that the following may trigger an episode;
- Infections and live vaccines
- Underlying autoimmune conditions such as Lupus
- Medical procedures, surgery and blood and marrow stem cell transplant and pancreatitis
- Medicines such as quinine, chemotherapy, ticlopidine, clopidogrel, cyclosporine A, hormone replacement therapy and estrogens
A medical history indicating any of the listed triggers, and a physical exam for symptoms, in combination with a complete blood count (CBC), lactate dehydrogenase level (LDH) and blood smear are used to determine a diagnosis of TTP. An ADAMTS 13 enzyme level test may be used, not to rule out, but, to help confirm the diagnosis. Importantly diagnosis and immediate treatment should not await the results of an ADAMTS 13 assay.
Hereditary TTP: Monthly prophylactic plasma is administered to patients to replenish and maintain adequate levels of functioning ADAMTS 13, the enzyme which the patient is unable to produce themselves.
Idiopathic & Secondary TTP: In some patients the steroid prednisone, has been used to slow the immune system and therefore the progression of this autoimmune disorder. The side effects of prednisone can be challenging and can include but not be limited to,
- increased appetite
- facial flushing
- mood swings
- vision changes
- moon face
- easy bruising
- unusual hair growth
In all cases of idiopathic or secondary adult TTP, plasma exchange is the basic treatment of choice. Plasma exchange involves the use of automated machinery which permits the removal of the patient's plasma and replacement with donor plasma during a 3 to 4 hour treatment. Plasma exchange both removes antibodies and replenishes normal plasma proteins. To treat TTP a series of daily or every other day plasma exchanges is used.
Other medications, such as Rituximab, and/or removal of the spleen are used when patients fail to achieve remission from first line therapy. Links to current TTP clinical trials can be found by clicking here.
Without treatment 95% of patients succumb to the disease, however; with treatment 80 - 90% of Idiopathic TTP patients achieve remission. Of these, about 30% will relapse and early detection of such a flare of the disease is critical to minimize the risk of death or irreversible injury to vital organs.
Printer Friendly Versions
Printer friendly PDF versions in English and professionally translated French, Italian, Portuguese, Punjabi, Russian and Simplified Chinese can be found HERE.
Note: German and Spanish translations are also available HERE, but they have been generously completed by volunteers and the accuracy of the content has not been validated. The content is intended for informational purposes only, and is not meant to substitute consultation from a recognized health professional.
Special thanks to Lisa Thielecke and Jaime Sanchez for volunteering their time to translate the content.
Please contact us if you are interested in translating content into additional languages.