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TTP — RACP Adult Medicine MCQ

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ModerateHaematologyTTPRACP Adult Medicine

A 30-year-old woman presents with severe thrombocytopenia (platelets 12 × 10⁹/L), microangiopathic haemolytic anaemia (schistocytes on film), fever, confusion, and renal impairment. ADAMTS13 activity is <5%. What is the most likely diagnosis?

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Correct answer: A — Thrombotic thrombocytopenic purpura

MAHA with severe thrombocytopenia and ADAMTS13 <10% is diagnostic of TTP. The classic pentad (thrombocytopenia, MAHA, fever, neurological symptoms, renal impairment) is only seen in ~5% of cases. Urgent plasma exchange must commence within 4–8 hours. Caplacizumab (anti-vWF nanobody) is now recommended in addition to PEX and immunosuppression.

Reference: Australian TMA Consensus 2018; BSH 2023 TTP Guidelines; ISTH 2025