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Thrombotic Thrombocytopenic Purpura

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

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—this is more common and is caused by other things
  • Inherited—present from birth

TTP needs care right away.

Causes

A certain enzyme breaks down a protein that stops excess clotting. Extra clotting can happen if there are problems with this enzyme, such as:
  • 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.

Risk Factors

TTP is more common in young adults, Black people, and women.

Health conditions that may be linked to TTP are:

  • Infection with a certain 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:
    • Drugs that block blood clot growth
    • Chemotherapy used to treat cancer
    • Medicines that suppress the immune system
    • Hormone therapies
    • Quinine found in some medicines and health products

Symptoms

A person with TTP may have:

  • Bruises or red spots
  • Lack of energy
  • Headache
  • Fever
  • 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

Diagnosis

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 Cells.

Red blood cell damage can cause fatigue and anemia.

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Treatment

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.

Prevention

There are no known guidelines to prevent this health problem.

RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

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; 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.

Contributors

  • James P. Cornell, MD
Last Updated:
2022-08-01

(C) Copyright 2023 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.