TTP Management — ESENeph MCQ

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HardAcute Kidney InjuryTTP ManagementESENeph

A 48-year-old woman with thrombotic thrombocytopenic purpura (TTP) presents with AKI (creatinine 250 umol/L), thrombocytopenia (20 x 10^9/L), MAHA (schistocytes), fever, and confusion. ADAMTS13 activity is <5%. What is the immediate treatment?

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Correct answer: E — Plasma exchange (TPE) plus Glucocorticoids plus Caplacizumab

TTP with ADAMTS13 <10% requires urgent plasma exchange (replaces ADAMTS13 and removes anti-ADAMTS13 antibodies), plus glucocorticoids (suppress autoantibody production), plus Caplacizumab (anti-von Willebrand factor nanobody that inhibits platelet microthrombi formation). The International Society on Thrombosis and Haemostasis (ISTH) 2020 guidelines recommend this triple therapy. Platelet transfusion is generally avoided as it may worsen thrombosis. Rituximab is added for refractory or relapsing disease.

Reference: ISTH 2020 – TTP Guidelines; BSH 2023 – TMA Guideline