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Make the connection between acquired thrombotic thrombocytopenic purpura (aTTP) and unexplained anaemia and thrombocytopenia before it is too late.

aTTP has a diverse presentation, but the most prominent presenting features are:1

A simple blood film can help to rule out certain thrombotic microangiopathies (TMAs).

aTTP patients are vulnerable and remain at risk of life-threatening complications, despite treatment with plasma exchange (PEX) + immunosupression (IMS).2–5

  • If left untreated, aTTP is rapidly fatal with an acute mortality rate of >90% and approximately half of aTTP deaths occur within 24 hours of presentation2,5
  • While patient survival rates are improved when treated with PEX+IMS alone vs. no treatment, mortality remains between 8–20%6

Performing a simple blood film to check for the presence of fragmented erythrocytes (schistocytes) confirms if a specialised assessment for aTTP is required and can potentially save a life.7

aTTP can be mistaken for many other more common diseases – rule it out in your differential diagnosis.2,8

The presence of microangiopathic haemolytic anaemia and thrombocytopenia is nonspecific, and differential diagnosis can include:8

  • Haemolytic uraemic syndrome (HUS)
  • Malignant hypertension
  • Disseminated intravascular coagulation
  • Sepsis
  • Pre-eclampsia

In all suspected cases of aTTP, refer patients to a specialist centre to confirm the diagnosis and initiate treatment.2

Confirming diagnosis of aTTP

A definitive diagnosis of aTTP can be confirmed using an ADAMTS13 assay at a specialised centre.2,8

If ADAMTS13 assay results are not available within 24 hours, the PLASMIC score or French score can be used to aid in the decision to initiate treatment by ‘predicting’ ADAMTS13 deficiency in suspected aTTP cases (adults only).9,10

  1. Veyradier A and Meyer D. J Thromb Haemost. 2005;3(11):2420–2427.
  2. Scully M, Hunt B, Benjamin S, et al. Br J Haematol. 2012;158:323–335.
  3. Goel R, King K, Takemoto C, et al. Transfusion. 2016;56(6):1451–1458.
  4. Rizvi MA, Vesely SK, George JN, et al. Transfusion. 2000;40:896–901.
  5. Scully M, Yarranton H, Liesner R, et al. Br J Haematol. 2008;142(5):819–826.
  6. Lester W, Begaj I, Ray D, et al. Poster presented at the 55th Annual Scientific Meeting of the British Society for Haematology, April 20–22, 2015.
  7. Rajan S. BMJ. 2016:1–32.
  8. Scully M, Cataland S, Coppo P, et al. J Thromb Hemost. 2017;15(2):312–322.
  9. Kremer Hovinga JA, Coppo P, Lämmle B, et al. Nat Rev Dis Primers. 2017;3:17020.
  10. Bendapudi PK, Hurwitz S, Fry A, et al. Lancet Haematol. 2017;4(4):e157–e164.

MAT-XU-2304823 (v1.0) Date of preparation: October 2023