The burden of thrombotic thrombocytopenic purpura (TTP)

Short- and long-term implications threaten patients with aTTP

Short-term implications

Mortality

  • Untreated, aTTP has an acute mortality rate of up to 90%1-3

  • Approximately 55% to 80% of deaths among patients with aTTP occur within 2 weeks of diagnosis4-6

  • Significantly higher rates of in-hospital mortality have also been reported in patients
    with TTP and CV complications (19.7% vs 4.1%)7

Health-related quality of life

  • Rates of depression (10% to 73%) are considerably higher in patients following an episode of TTP compared with both healthy individuals and reference populations8-12

Morbidity

  • Most common complications in patients hospitalized with TTP are CV complications (25%), such as stroke (10%), heart failure (8%), and acute coronary syndrome (6%)7

Economic burden

  • Short-term treatment of acute thromboembolism such as stroke, acute MI, or TIA requires multiple healthcare resources, including hospitalization with diagnostic tests (eg, CT scans), administration of thrombolytic and/or other drugs, admittance to intensive care/critical care unit, and/or ongoing specialist nursing care
    (eg, physiotherapy, speech therapy)13-15

An increased time at risk of microvascular thrombosis can lead to irreversible organ damage to the heart, brain, and kidneys16

Long-term implications

Mortality

  • 23-fold increase in mortality was observed in patients who did not reach a platelet recovery rate of 5 × 109/L per day by day 3 of PEX17

  • Inability to normalize platelet count over 7 days of PEX was associated with a significantly increased risk of death18

Health-related quality of life

  • Long-term outcomes are driven by the consequences of platelet aggregation, leading to systemic microvascular thrombosis7,19

Morbidity

  • Recovery following an acute episode of aTTP is not a resolution of symptoms but the beginning of a long-term morbidity burden: hypertension, stroke, and depression requiring pharmacological treatment were significantly greater for TTP survivors versus the age- and sex-matched US population8,9,20

Economic burden

  • Other long-term consequences that have been reported include neurocognitive impairment, depression, hypertension, headaches, and SLE; all require healthcare resources on an ongoing basis11,20

Long-term morbidity leads to suboptimal health-related quality of life, in both mental (anxiety, depression) and physical functioning, and potentially reduces life expectancy8,20

Knowing the clinical science behind aTTP helps explain the severe effects of the disease.

See TTP pathophysiology

The ISTH TTP Guidelines can help inform diagnosis and your treatment approach.

See the Guidelines

aTTP=acquired thrombotic thrombocytopenic purpura; CT=computed tomography; CV=cardiovascular; MI=myocardial infarction; PEX=plasma exchange; SLE=systemic lupus erythematosus; TIA=transient ischemic attack; TTP=thrombotic thrombocytopenic purpura.

References: 1. Scully M, Hunt BJ, Benjamin S, et al; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323-335. doi:10.1111/j.1365-2141.2012.09167.x 2. Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115(8):1500-1511. doi:10.1182/blood-2009-09-243790 3. Sayani FA, Abrams CS. How I treat refractory thrombotic thrombocytopenic purpura. Blood. 2015;125(25):3860-3867. doi:10.1182/blood-2014-11-551580 4. Goel R, King KE, Takemoto CM, Ness PM, Tobian AAR. Prognostic risk-stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion. 2016;56(6):1451-1458. doi:10.1111/trf.13586 5. Benhamou Y, Assié C, Boelle P-Y, et al. Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. Haematologica. 2012;97(8):1181-1186. doi:10.3324/haematol.2011.049676 6. Chaturvedi S, Bhatia N. Predictors of survival in thrombotic thrombocytopenic purpura. Haematologica. 2013;98(5):e58. doi:10.3324/haematol.2012.079400 7. Balasubramaniyam N, Yandrapalli S, Kolte D, Pemmasani G, Janakiram M, Frishman WH. Cardiovascular complications and their association with mortality in patients with thrombotic thrombocytopenic purpura. Am J Med. 2021;134(2):e89-e97. doi:10.1016/j.amjmed.2020.06.020 8. Chaturvedi S, Abbas H, McCrae KR. Increased morbidity during long-term follow-up of survivors of thrombotic thrombocytopenic purpura. Am J Hematol. 2015;90(10):E208. doi:10.1002/ajh.24138 9. Falter T, Schmitt V, Herold S, et al. Depression and cognitive deficits as long-term consequences of thrombotic thrombocytopenic purpura. Transfusion. 2017;57(5):1152-1162. doi:10.1111/ trf.14060 10. Han B, Page EE, Stewart LM, et al. Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura. Am J Hematol. 2015;90(8):709-714. doi:10.1002/ajh.24060 11. Riva S, Mancini I, Maino A, et al. Long-term neuropsychological sequelae, emotional wellbeing and quality of life in patients with acquired thrombotic thrombocytopenic purpura. Haematologica. 2020;105(7):1957-1962. doi:10.3324/haematol.2019.226423 12. Alwan F, Mahdi D, Tayabali S, et al. Cerebral MRI findings predict the risk of cognitive impairment in thrombotic thrombocytopenic purpura. Br J Haematol. 2020;191(5):868-874. doi:10.1111/bjh.17126 13. Hughes PA. Comprehensive care of adults with acute ischemic stroke. Crit Care Nurs Clin N Amer. 2011;23(4):661-675. doi:10.1016/j.ccell.2011.08.009 14. Reed GW, Rossi JE, Cannon CP. Acute myocardial infarction. Lancet. 2017;389(10065):197-210. doi:10.1016/s0140-6736(16)30677-8 15. Lau CS, Mak A. The socioeconomic burden of SLE. Nat Rev Rheumatol. 2009;5(7):400-404. doi:10.1038/nrrheum.2009.106 16. Thejeel B, Garg AX, Clark WF, et al. Long-term outcomes of thrombotic microangiopathy treated with plasma exchange: a systematic review. Am J Hematol. 2016;91(6):623-630 doi:10.1002/ajh.24339 17. Liu C, Kallogjeri D, Dynis M, Grossman BJ. Platelet recovery rate during plasma exchange predicts early and late responses in patients with thrombotic thrombocytopenic purpura. Transfusion. 2013(5):1096-1107. doi:10.1111/j.1537-2995.2012.03857.x 18. Staley EM, Cao W, Pham HP, et al. Clinical factors and biomarkers predict outcome in patients with immune-mediated thrombotic thrombocytopenic purpura. Haematologica. 2019;104(1):166-175. doi:10.3324/haematol.2018.198275 19. Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017;3:17020. doi:10.1038/nrdp.2017.20 20. 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(12):2023-2029. doi:10.1182/blood-2013-04-496752