Shruti Chaturvedi, MBBS, MS, Assistant Professor of Medicine at Johns Hopkins provides an overview of thrombotic thrombocytopenic purpura (TTP).
TTP is a rare hematologic condition due to reduced activity of ADAMTS13 enzyme. The reduced activity is either due to a person developing antibodies to ADAMTS13 (acquired TTP) or they have a genetic mutation to reduces the production of the enzyme (inherited TTP).
As Dr. Chaturvedi explains in this interview, TTP can be life threatening if not diagnosed and treated properly. A person with TTP usually presents at the emergency department or clinic with severe symptoms that warrant monitoring while blood tests are run. The definitive diagnosis will be low ADAMTS13 activity but not all medical centers have that can be readily available. As such, three pathways can help lead to a proper diagnosis, based on the availability of testing.
Once diagnosed, or suspected, treatment should begin with plasma exchange as well as the possible use of corticosteroids and rituximab. The newly approved orphan drug, caplacizumab, is also recommended. Caplacizumab blocks platelet-von Willebrand factor interactions and thereby prevents the formation of microvascular thrombosis in small vessels.
To learn more about TTP and other hematologic conditions, click here.