Thrombotic Thrombocytopenic Purpura
(TTP; Moschcowitz Syndrome)
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. It makes blood clot in small blood vessels all over the body. This can block the flow of blood to major organs. It also uses up the platelets needed to help blood clot. This makes bleeding more likely and can lead to bruises.
There are two types:
- Acquired—this is more common and is caused by other things
- Inherited—present from birth
TTP needs care right away.
- There are low levels of it
- It is not working as it should
- It is delayed
People with acquired TTP have normal enzymes, but their activity is blocked or slowed by other things. A medical treatment or infection may trigger the immune system to start blocking the enzymes from working.
Inherited TTP is caused by a problem with a certain gene. The problem prevents the body from making the enzyme or making an enzyme that works.
TTP is more common in young adults, Black people, and women.
Health conditions that may be linked to TTP are:
Medical procedures or treatments that are linked to TTP are:
A person with TTP may have:
- Bruises or red spots
- Lack of energy
- A fast heartbeat
- Problems breathing
- Belly pain, nausea, or vomiting
- Bloody, loose stools or blood in the urine
- Yellowing of the skin and whites of the eyes
- Changes in behavior or mental state
The doctor will ask about symptoms and health history. A physical exam will be done. A doctor who treats blood disorders may need to be seen.
Blood tests will be done to look for signs of TTP, such as a low number of platelets and red blood cells.
More tests may be done to look for a cause.
Red blood cell damage can cause fatigue and anemia.
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The goal of treatment is to prevent damage to major organs or serious problems such as stroke. A person may have TTP once or have flare ups that come and go. Emergency care is needed. Ways to treat TTP are:
- Plasma therapy, such as:
- Injecting plasma to add missing enzymes
- Removing harmful antibodies from a person's plasma and adding missing enzymes
- Medicines to:
- Slow damage to red blood cells
- Tell bone marrow to make more platelets
- Suppress the immune system
Cells in the spleen make the antibodies that block how the enzyme works. People with acquired TTP, those who do not get better with medicine, and those who have flare ups may need surgery to remove the spleen.
There are no known guidelines to prevent this health problem.
- Scully, M., Cataland, S., et al; International Working Group for Thrombotic Thrombocytopenic Purpura. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017; 15 (2): 312-322.
- Thrombotic thrombocytopenic purpura. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health/thrombotic-thrombocytopenic-purpura.
- Thrombotic thrombocytopenic purpura (TTP). EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/thrombotic-thrombocytopenic-purpura-ttp.
- Thrombotic thrombocytopenic purpura (TTP). Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/thrombotic-thrombocytopenic-purpura-ttp.
- James P. Cornell, MD
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