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 activity3
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
- Arterial hypertension
Diagnosing and referring quickly is key to
reducing the high rate of mortality.1,7,8,12
- Joly B, Coppo P and Veyradier A. Blood. 2017;129(21):2836–2846.
- Alwan F, Vendramin C, Vanhoorelbeke K, et al. Blood. 2017;130(4):466–471.
- Scully M, Cataland SR, Peyvandi F, et al. NEJM. 2019;380:335–346.
- Zheng XL, Vesely SK, Cataland SR, et al. J Thromb Haemost. 2020;18(10):2496–2502.
- Sadler JE. Blood. 2008;112(1):11–18.
- Peyvandi F, Garagiola I and Baronciani L. Blood Transfus. 2021;9(Suppl 2):s3–s8.
- Scully M, Hunt BJ, Benjamin S, et al. Br J Haematol. 2012;158(3):323–335.
- Scully M, Yarranton H, Liesner R, et al. Br J Haematol. 2008;142(5):819–826.
- Han B, Page EE, Stewart LM, et al. Am J Hematol. 2015;90(8):709–714.
- Deford CC, Reese JA, Schwartz LH, et al. Blood. 2013;122(12):2023–2029.
- Clinical Review Report: Caplacizumab (Cablivi). CADTH Common Drug Review. 2020. Available at: https://www.ncbi.nlm.nih.gov/books/NBK567585 Accessed April 2022.
- Veyradier A and Meyer D. J Thromb Haemost. 2005;3(11):2420–2427.
ADAMTS13, a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13; aTTP, acquired thrombotic thrombocytopenic purpura; BSH, British Society for Haematology; GP, glycoprotein; iTTP, immune-mediated thrombotic thrombocytopenic purpura; LDH, lactate dehydrogenase; PEX, plasma exchange; TTP, thrombotic thrombocytopenic purpura; ULvWF, ultra-large von Willebrand factor; vWF, von Willebrand factor.
Date of preparation: April 2022