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Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an ultra-rare, life-threatening, thrombotic microangiopathy manifested by microvascular occlusions and consequent thrombocytopenia, haemolytic anaemia and organ ischaemia.1

TTP primarily affects young, healthy adults.2

Types of TTP

Acquired TTP (Also known as immune-mediated thrombotic thrombocytopenic purpura (iTTP))3

  • The most common form of TTP4
  • Caused by autoantibody inhibition of ADAMTS13 activity

Hereditary TTP (also known as congenital TTP, inherited TTP, familial TTP, or Upshaw–Schulman syndrome)4

  • Very rare form of TTP4
  • Caused by mutations in the ADAMTS13 gene4

Approximately 95% of TTP cases are aTTP.4

Effects of microthrombi in aTTP: The serious consequences of microthrombi and their impact on key organs.

The most commonly affected organs in aTTP are:7

If left untreated, aTTP is rapidly fatal with an acute mortality rate of >90%.7 Approximately half of aTTP deaths occur within 24 hours of presentation.8

aTTP has a long-term effect on patients

In addition to the acute risks of the disease, patients experiencing an episode of aTTP may suffer long-term consequences such as:9–11

  • Cognitive deficits
  • Depression
  • Arterial hypertension

Diagnosing and referring quickly is key to reducing the high rate of mortality.1,7,8,12

  1. Joly B, Coppo P and Veyradier A. Blood. 2017;129(21):2836–2846. 
  2. Alwan F, Vendramin C, Vanhoorelbeke K, et al. Blood. 2017;130(4):466–471. 
  3. Scully M, Cataland SR, Peyvandi F, et al. NEJM. 2019;380:335–346. 
  4. Zheng XL, Vesely SK, Cataland SR, et al. J Thromb Haemost. 2020;18(10):2496–2502.  
  5. Sadler JE. Blood. 2008;112(1):11–18. 
  6. Peyvandi F, Garagiola I and Baronciani L. Blood Transfus. 2021;9(Suppl 2):s3–s8. 
  7. Scully M, Hunt BJ, Benjamin S, et al. Br J Haematol. 2012;158(3):323–335. 
  8. Scully M, Yarranton H, Liesner R, et al. Br J Haematol. 2008;142(5):819–826. 
  9. Han B, Page EE, Stewart LM, et al. Am J Hematol. 2015;90(8):709–714. 
  10. Deford CC, Reese JA, Schwartz LH, et al. Blood. 2013;122(12):2023–2029. 
  11. Clinical Review Report: Caplacizumab (Cablivi). CADTH Common Drug Review. 2020. Available at: Accessed November 2022. 
  12. Veyradier A and Meyer D. J Thromb Haemost. 2005;3(11):2420–2427.

MAT-XU-2304824 (v1.0) Date of preparation: October 2023