Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. In TTP, blood clots form in small blood vessels throughout the body. The clots can block the flow blood to the body's organs, such as the brain, kidneys, and heart. The increased clotting that occurs in TTP also uses up platelets in the blood. Platelets help form blood clots. With fewer platelets available in the blood, bleeding problems can occur. People who have TTP may bleed inside their bodies, underneath the skin, or from the surface of the skin.

There are Two types of TTP:

  • In inherited TTP, the ADAMTS13 gene is faulty and doesn't prompt the body to make a normal ADAMTS13 enzyme. As a result, enzyme activity is lacking or changed.
  • Acquired TTP is the more common type of the disorder. "Acquired" means you aren't born with the disorder, but you develop it. This type of TTP mostly occurs in adults, but it can affect children.

Test Code Test Name Ordering recommendations
0030056 ADAMTS13 Activity Assists in diagnosing congenital or inherited thrombotic thrombocytopenic purpura (TTP).
3000182 ADAMTS13 Antibody Assist in distinguishing between inherited and acquired forms of thrombotic thrombocytopenic purpura (TTP). Not recommended as initial test for identification of autoantibodies to ADAMTS13, since the ADAMTS13 antibody test is less specific for acquired TTP than ADAMTS13 Inhibitor (3000228). Order when acquired TTP is suspected but antibodies are not identified by the ADAMTS13 inhibitor test.
3000228 ADAMTS13 Inhibitor Assist in distinguishing between inherited and acquired forms of thrombotic thrombocytopenic purpura (TTP). Recommended initial test for the identification of autoantibodies to ADAMTS13, since the ADAMS13 inhibitor test is more specific for acquired TTP than the ADAMTS13 antibody test. If suspicion for TTP remains after a negative result, ADAMTS13 Antibody (3000182) is recommended.
3000239 ADAMTS13 Reflex Panel Reflexive panel to assist in diagnosis of thrombotic thrombocytopenic purpura (TTP) and in distinguishing between inherited and acquired forms of TTP.

Related Topics