skip to main content

TTP Coexisting SLE Management — ESENeph MCQ

Instant feedback + full explanation. One question, done properly.

HardGlomerulonephritisTTP Coexisting SLE ManagementESENeph

An adult has microangiopathic haemolytic anaemia, severe thrombocytopenia, neurological symptoms and ADAMTS13 activity below 10%, confirming acute acquired TTP. Which regimen follows NICE?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: DCaplacizumab plus plasma exchange and immunosuppression

The best answer is “Caplacizumab plus plasma exchange and immunosuppression”. NICE recommends caplacizumab with plasma exchange and immunosuppression for acute acquired TTP. Plasma exchange is started urgently, while immunosuppression addresses the anti-ADAMTS13 autoantibody. “Use caplacizumab alone and omit plasma exchange” is less appropriate because caplacizumab is an adjunct and does not replace rapid antibody removal and ADAMTS13 replacement “Wait for spontaneous platelet recovery before treatment” is less appropriate because acquired TTP is life-threatening and requires immediate therapy “Treat with platelet transfusion as definitive routine therapy” is less appropriate because platelet transfusion can worsen microvascular thrombosis and does not treat the mechanism “Use maintenance haemodialysis as the primary TTP treatment” is less appropriate because dialysis supports severe kidney failure but does not treat acquired TTP

Reference: NICE TA667: caplacizumab for acquired TTP: https://www.nice.org.uk/guidance/ta667/chapter/1-Recommendations