Acquired TTP: Treatment of refractory or relapsed disease
- 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, thrombocytopenia, and microangiopathic hemolytic anemia. In addition, some patients may have neurologic abnormalities, mild renal insufficiency, or low-grade fever.
The use of plasma exchange therapy since the early 1990s has converted TTP from an almost uniformly fatal condition to a treatable disease. However, 30 to 40 percent of patients with TTP will have a transient, incomplete, or no response to treatment with plasma exchange and glucocorticoids (ie, refractory disease). Among patients whose disease goes into remission, another 20 percent will have another acute episode of TTP (ie, relapsed disease). (See 'Terminology' below.)
This topic reviews our approach to the patient with refractory or relapsed acquired autoimmune TTP (TTP caused by an autoantibody to ADAMTS13).
Our approach to the diagnosis of acquired TTP, the initial management of acquired TTP, and the management during remission are presented in detail separately. (See "Acquired TTP: Clinical manifestations and diagnosis" and "Acquired TTP: Initial treatment" and "Acquired TTP: Management following recovery from an acute episode and during remission".)
Separate topic reviews also discuss management of related primary thrombotic microangiopathies:
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- REFRACTORY DISEASE
- Overview of approach (refractory disease)
- Re-evaluation of the diagnosis
- - Other possible diagnoses
- - Role of ADAMTS13 retesting
- Plasma exchange
- High-dose glucocorticoids
- POOR RESPONSE TO HIGH-DOSE GLUCOCORTICOIDS AND RITUXIMAB
- Approach to additional therapies
- Mycophenolate mofetil
- Overview of relapse
- First relapse
- Multiple relapses
- SUMMARY AND RECOMMENDATIONS