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 at 10–20%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
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
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
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
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.