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Thrombotic thrombocytopenic purpura

What's new

The International Society on Thrombosis and Haemostasis (ISTH) has published a focused guideline for the management of immune thrombotic thrombocytopenic purpura (iTTP) and congenital thrombotic thrombocytopenic purpura (cTTP). For the management of an acute event in iTTP, treatment includes therapeutic plasma exchange and corticosteroids, with the suggested addition of rituximab and caplacizumab. Rituximab is also suggested for maintenance of remission in asymptomatic patients with low ADAMTS-13 activity. For patients with cTTP who are in remission, prophylaxis with plasma infusion is suggested over a watch-and-wait strategy, and recombinant ADAMTS-13 is recommended over plasma infusion to prevent acute episodes. .

Guidelines

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 International Society on Thrombosis and Haemostasis (ISTH 2025,2020), the British Society for Haematology (BSH 2023), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), the European Dialysis and Transplant Association (ERA-EDTA/EULAR 2020), the Blood Coagulation Abnormalities ...
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Diagnostic investigations

Initial evaluation
As per BSH 2023 guidelines:
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

Medical management

General principles
As per BSH 2023 guidelines:
Treat TTP as a medical emergency with time-critical transfer to a dedicated treatment center.
A
Obtain a pretransfer review by an appropriately skilled medical team. Consider performing intubation in clinically unstable patients.
B
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  • Management of an acute event (corticosteroids)

  • Management of an acute event (rituximab)

  • Management of an acute event (caplacizumab)

  • Management of refractory disease

  • Maintenance of remission

  • Management of relapse

  • Antiplatelet therapy

Inpatient care

Thromboprophylaxis: as per BSH 2023 guidelines, administer thromboprophylaxis in all hospitalized/immobilized patients once platelet counts are ≥ 50×10⁹/L, even when treated with caplacizumab.
B
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Nonpharmacologic interventions

Folate supplementation: as per BCSH 2012 guidelines, offer folate supplements in patients with active hemolysis.
A
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  • Psychosocial support

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.
A
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  • Platelet transfusion

  • RBC transfusion

Surgical interventions

Splenectomy: as per BSH 2023 guidelines, consider performing splenectomy as an option in patients with refractory or relapsing immune-mediated TTP.
C
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Specific circumstances

Pregnant patients: as per ISTH 2025 guidelines, initiate prophylactic treatment in pregnant patients with immune-mediated TTP with decreased plasma ADAMTS-13 activity but no clinical signs or symptoms.
B
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  • Patients with HIV

  • Patients with congenital TTP (management)

  • Patients with lupus nephritis-associated TMA

  • Patients with other thrombotic microangiopathies

  • Patients with congenital TTP (diagnosis)

Patient education

General counseling: as per BCSH 2012 guidelines, counsel patients and provide with verbal and written information about symptoms, signs and risk of relapse before discharge.
A
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Follow-up and surveillance

Laboratory follow-up: as per BSH 2023 guidelines, obtain lifelong follow-up, including ADAMTS13 assay monitoring, in patients with TTP.
B
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