Acquired TTP: Management following recovery from an acute episode and during remission
- 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
Most patients (85 to 90 percent) with acquired autoimmune thrombotic thrombocytopenic purpura (TTP) are treated effectively with plasma exchange (PEX), glucocorticoids, and, for many patients, rituximab. However, relapse following remission is an important concern. Therefore, all patients who have recovered from an episode of TTP require lifelong monitoring and rapid intervention at the first sign of a relapse, and some may benefit from prophylactic immunosuppressive treatment. Additionally, patients require continued surveillance for a variety of important long-term health problems related to TTP.
This topic review discusses our approach to the management of a patient who has recovered from an acute episode of acquired TTP, including routine monitoring, evaluation for symptoms of relapse, prophylaxis, and management of long-term morbidities.
Separate topic reviews discuss other aspects of care for patients with acquired, autoimmune TTP (ie, TTP due to an autoantibody against ADAMTS13):
●Diagnosis – (See "Acquired TTP: Clinical manifestations and diagnosis".)
●Treatment of an acute episode – (See "Acquired TTP: Initial treatment".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Froissart A, Buffet M, Veyradier A, et al. Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med 2012; 40:104.
- Page EE, Kremer Hovinga JA, Terrell DR, et al. Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Advances 2017; 1:590.
- Hie M, Gay J, Galicier L, et al. Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura. Blood 2014; 124:204.
- Westwood JP, Thomas M, Alwan F, et al. Rituximab prophylaxis to prevent thrombotic thrombocytopenic purpura relapse: Outcome and evaluation of dosing regimens. Blood Advances 2017; 1:1159.
- Lim W, Vesely SK, George JN. The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura. Blood 2015; 125:1526.
- van Vollenhoven RF, Emery P, Bingham CO 3rd, et al. Long-term safety of rituximab in rheumatoid arthritis: 9.5-year follow-up of the global clinical trial programme with a focus on adverse events of interest in RA patients. Ann Rheum Dis 2013; 72:1496.
- Kremer Hovinga JA, Vesely SK, Terrell DR, et al. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 2010; 115:1500.
- Page EE, Kremer Hovinga JA, Terrell DR, et al. Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura. Blood 2016; 127:3092.
- Griffin D, Al-Nouri ZL, Muthurajah D, et al. First symptoms in patients with thrombotic thrombocytopenic purpura: what are they and when do they occur? Transfusion 2013; 53:235.
- Nazi I, Kelton JG, Larché M, et al. The effect of rituximab on vaccine responses in patients with immune thrombocytopenia. Blood 2013; 122:1946.
- Chaturvedi S, Abbas H, McCrae KR. Increased morbidity during long-term follow-up of survivors of thrombotic thrombocytopenic purpura. Am J Hematol 2015; 90:E208.
- Deford CC, Reese JA, Schwartz LH, et al. Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura. Blood 2013; 122:2023.
- Little DJ, Reese JA, Vesely SK, George JN. Increased urinary albumin excretion following recovery from thrombotic thrombocytopenic purpura due to acquired ADAMTS13 deficiency. Am J Kidney Dis 2014; 64:317.
- Han B, Page EE, Stewart LM, et al. Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura. Am J Hematol 2015; 90:709.
- Kennedy AS, Lewis QF, Scott JG, et al. Cognitive deficits after recovery from thrombotic thrombocytopenic purpura. Transfusion 2009; 49:1092.
- Howard MA, Duvall D, Terrell DR, et al. A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): The six-year experience of the Oklahoma TTP-HUS Study Group. J Clin Apher 2003; 18:16.
- Ambadwar P, Duvall D, Wolf NJ, et al. Support groups for patients who have recovered from thrombotic thrombocytopenic purpura. J Clin Apher 2008; 23:168.
- Jiang Y, McIntosh JJ, Reese JA, et al. Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura. Blood 2014; 123:1674.
- Scully M, Thomas M, Underwood M, et al. Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood 2014; 124:211.
- MONITORING AND THERAPY AFTER RESPONSE TO TREATMENT
- First week
- First month
- First year
- Subsequent years
- ADAMTS13 measurements
- Role of rituximab to reduce relapse risk
- - Overview of our decision process
- - Rituximab dosing and risks
- Risk of relapse
- Symptoms of disease relapse
- Diagnostic evaluation for suspected relapse
- Urgency of intervention
- ROUTINE MEDICAL CARE DURING REMISSION
- Evaluation for complications of TTP
- - Hypertension and cardiovascular disease
- - Renal impairment
- - Depression
- - Minor cognitive impairment
- - Systemic lupus erythematosus
- PREGNANCY AFTER AN EPISODE OF TTP
- SUMMARY AND RECOMMENDATIONS