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Thrombotic thrombocytopenic purpura

Key sources
The following summarized guidelines for the evaluation and management of thrombotic thrombocytopenic purpura are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2023), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), the International Society on Thrombosis and Haemostasis (ISTH 2020), the European Dialysis and Transplant Association (ERA-EDTA/EULAR 2020), the Blood Coagulation Abnormalities Research Team (BCART 2017), and the British Committee for Standards In Haematology (BCSH 2012).
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Guidelines

1.Diagnostic investigations

Initial evaluation
Diagnose TTP based on clinical history, physical examination, and routine laboratory tests, including blood film.
A
Obtain serological tests for HIV, HBV, HCV, autoantibody screen, and when appropriate, a pregnancy test at presentation.
A
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  • ADAMTS13 activity and anti-ADAMTS13 antibodies

2.Medical management

General principles
Treat TTP as a medical emergency with time-critical transfer to a dedicated treatment center.
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Obtain a pretransfer review by an appropriately skilled medical team. Consider performing intubation in clinically unstable patients.
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  • Corticosteroids

  • Caplacizumab

  • Rituximab

  • Maintenance of remission

  • Antiplatelet therapy

3.Inpatient care

Thromboprophylaxis: administer thromboprophylaxis in all hospitalized/immobilized patients once platelet counts are ≥ 50×10⁹/L, even when treated with caplacizumab.
B

4.Nonpharmacologic interventions

Folate supplementation: offer folate supplements in patients with active hemolysis.
A

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  • Psychosocial support

5.Therapeutic procedures

Plasma exchange: as per BSH 2023 guidelines, perform plasma exchange within 4-8 hours from referral of a suspected diagnosis of TTP and transfer.
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  • Platelet transfusion

  • RBC transfusion

6.Surgical interventions

Splenectomy: consider performing splenectomy as an option in patients with refractory or relapsing immune-mediated TTP.
C

7.Specific circumstances

Pregnant patients: as per BSH 2023 guidelines, normalize ADAMTS13 activity before pregnancy in patients with immune-mediated TTP.
B
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  • Patients with HIV

  • Patients with congenital TTP

  • Patients with lupus nephritis-associated TMA

  • Patients with other thrombotic microangiopathies

8.Patient education

General counseling: counsel patients and provide with verbal and written information about symptoms, signs and risk of relapse before discharge.
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9.Follow-up and surveillance

Serial laboratory assessment: obtain lifelong follow-up, including ADAMTS13 assay monitoring, in patients with TTP.
B

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  • Management of refractory disease

  • Management of relapse