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TTP in SLE — SCE Rheumatology MCQ

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HardRheumatology EmergenciesTTP in SLESCE Rheumatology

A woman with SLE has confusion, platelets 25 ×10^9/L, schistocytes, high LDH and ADAMTS13 activity below 5%. What is the urgent treatment approach?

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Correct answer: B — Start plasma exchange, corticosteroids and caplacizumab for TTP

The best answer is “Start plasma exchange, corticosteroids and caplacizumab for TTP”. Severe ADAMTS13 deficiency with microangiopathic haemolysis and thrombocytopenia supports immune TTP, a medical emergency requiring immediate specialist therapy. Anticoagulation alone does not treat TTP, routine platelet transfusion can be harmful, eculizumab targets complement-mediated HUS, and observation risks fatal organ injury.

Reference: British Society for Haematology thrombotic thrombocytopenic purpura guideline: https://b-s-h.org.uk/guidelines/guidelines/diagnosis-and-management-of-thrombotic-thrombocytopenic-purpura-and-thrombotic-microangiopathies