Diagnosis

 

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

 

prominent presenting features

 

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 at 1020%2

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.6


BSH webinar: 16-year-old female case study

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Further resources

video

Expert opinion: signs and symptoms

video

Expert opinion: 24-year-old female case study

Differential diagnosis of TMAs thumbnail

Differential diagnosis of TMAs

 


aTTP can be mistaken for many other more common diseases – rule it out in your differential diagnosis.2,7

 

The presence of microangiopathic haemolytic anaemia and thrombocytopenia is nonspecific, and differential diagnosis can include:7

  • 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

 

Expert opinion: diagnosis of aTTP

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Further resources

video

BSH webinar: 16-year-old female case study

Differential diagnosis of TMAs thumbnail

Differential Diagnosis of TMAs

video

BSH webinar: 48-year-old male case study

 

Confirming diagnosis of aTTP.

 

A definitive diagnosis of aTTP can be confirmed using an ADAMTS13 assay at a specialised centre.2,7

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).8,9

 

Predicting severe ADAMTS13 deficiency in aTTP

Predicting severe ADAMTS13

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Further resources

Speed of Treatment

BSH webinar: speed of treatment initiation

Resource - scoring system in aTTP

The PLASMIC score in aTTP

Diagnosis of aTTP video thumbnail

Expert opinion: diagnosis of aTTP

Be ready for aTTP.

TREATMENT

ADAMTS13, a disintegrin and metaloprotease with thrombospondin type 1 motif, member 13; aTTP, acquired thrombotic thrombocytopenic purpura; BSH, British Society for Haematology; HUS, haemolytic uraemic syndrome; IMS, immunosupression; PEX, plasma exchange; TMA, thrombotic microangiopathy.


References

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. Rajan S. BMJ. 2016:1–32.
7. Scully M, Cataland S, Coppo P, et al. J Thromb Hemost. 2017;15(2):312–322.
8. Kremer Hovinga JA, Coppo P, Lämmle B, et al. Nat Rev Dis Primers. 2017;3:17020.
9. Bendapudi PK, Hurwitz S, Fry A, et al. Lancet Haematol. 2017;4(4):e157–e164.

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