Acquired TTP: Clinical manifestations and diagnosis
- James N George, MD
James N George, MD
- Professor of Medicine
- University of Oklahoma Health Sciences Center
- Adam Cuker, MD, MS
Adam Cuker, MD, MS
- Assistant Professor of Medicine, Assistant Professor of Pathology & Laboratory Medicine
- Perelman School of Medicine University of Pennsylvania
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia, and sometimes organ damage. TTP is a medical emergency that is almost always fatal if appropriate treatment is not initiated promptly. With appropriate treatment, survival rates of up to 90 percent are possible.
TTP can be acquired, due to an autoantibody inhibitor, or hereditary, due to inherited mutations in ADAMTS13. This topic reviews the clinical manifestations and our approach to the diagnosis of acquired autoimmune TTP. Our approach to evaluating patients with other suspected thrombotic microangiopathies (including hereditary TTP) is presented in separate topic reviews:
●Suspected TTP or hemolytic uremic syndrome (HUS), diagnosis unclear – (see "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)")
●Suspected hereditary TTP – (see "Hereditary thrombotic thrombocytopenic purpura (TTP)")
●Suspected drug-induced thrombotic microangiopathy (also called drug-induced TTP) – (see "Drug-induced thrombotic microangiopathy")
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- TERMINOLOGY AND DISEASE DEFINITIONS
- CLINICAL AND LABORATORY FINDINGS
- Overview of presentation
- - MAHA and thrombocytopenia
- - Neurologic and other organ involvement
- - Reduced ADAMTS13 activity
- Atypical presentations
- DIAGNOSTIC TESTING
- Overview of our approach
- Diagnostic evaluation
- ADAMTS13 testing
- - ADAMTS13 activity
- - Assay considerations
- - ADAMTS13 inhibitor
- Presumptive diagnosis
- Confirmed diagnosis
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS