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Thrombotic Thrombocytopenic Purpura

(TTP; Moschcowitz Syndrome)

Definition

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—more common, resulting from other causes
  • Inherited—present from birth
TTP needs care right away.

Causes

A specific enzyme breaks down a protein that prevents excess clotting. Low levels of this enzyme, faulty enzymes, or delay with the enzyme, leads to excessive clotting.
People with acquired TTP have normal enzymes, but their activity is blocked or slowed by outside factors. It may be triggered by medical treatments or infections, especially those that affect the immune system. Normally, the immune system creates antibodies to fight foreign matter in the body like germs. However, sometimes these antibodies can affect healthy tissue. With TTP, the antibodies block the action of the enzymes.
Inherited TTP is caused by a defect of a specific gene. The defect prevents the body from creating the enzyme or creating effective enzymes.

Risk Factors

TTP is more common in women, Black people, and young adults.
Health conditions that may be linked to TTP are:
  • Infection with a specific bacterium
  • Cancer
  • Pregnancy
  • Lupus
  • Other infections, such as HIV
Medical procedures or treatments that are linked to TTP are:
  • Bone marrow or stem cell transplants
  • Medicines, such as:
    • Antiplatelet or anticoagulant drugs that block blood clot growth
    • Chemotherapy used to treat cancer
    • Medicines that suppress the immune system
    • Hormone therapies
    • Quinine found in certain medicines and health products

Symptoms

Problems may be:
  • Bruises or red spots
  • Lack of energy
  • Headache
  • Fever
  • A fast heartbeat
  • Problems breathing
  • Belly pain
  • Nausea and vomiting
  • Bloody, loose stools
  • Blood in the urine
  • Yellowing of the skin and whites of the eyes
  • Changes in behavior or mental state

Diagnosis

The doctor will ask about your symptoms and health history. A physical exam will be done. You may need to see a doctor who treats blood disorders.
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 Cells
Nucleus factsheet image
Red blood cell damage can cause fatigue and anemia.
Copyright © Nucleus Medical Media, Inc.

Treatment

A person may have TTP once or have repeated flare ups. Emergency care is needed to prevent damage to major organs and serious problems such as stroke. Choices are:
  • Plasma therapy, such as:
    • Plasma infusion to add missing enzymes
    • Plasma exchange to remove harmful antibodies and add missing enzymes
  • Medicines to:
    • Slow damage to red blood cells
    • Stimulate bone marrow to make more platelets
    • Suppress the immune system
Cells in the spleen make the antibodies that block the enzyme activity. 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.

Prevention

There are no known guidelines to prevent this health problem.

RESOURCES

American Society of Hematology
http://www.hematology.org
National Heart, Lung, and Blood Institute
http://www.nhlbi.nih.gov

CANADIAN RESOURCES

Canadian Blood Services
https://www.blood.ca
Public Health Agency of Canada
http://www.phac-aspc.gc.ca

References

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 Feb;15(2):312-322.
Thrombotic thrombocytopenic purpura. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/thrombotic-thrombocytopenic-purpura. Accessed February 12, 2021.
Thrombotic thrombocytopenic purpura (TTP). EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/thrombotic-thrombocytopenic-purpura-ttp. Accessed February 12, 2021.
Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS). Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/thrombotic-thrombocytopenic-purpura-ttp-and-hemolytic-uremic-syndrome-hus. Accessed February 12, 2021.

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