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Thrombotic thrombocytopenic purpura — ABIM Board MCQ

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HardHematology/OncologyThrombotic thrombocytopenic purpuraABIM Board

A 43-year-old has confusion, platelets 12,000/mm3, microangiopathic hemolytic anemia, creatinine 1.9 mg/dL and normal PT/aPTT. ADAMTS13 activity has been sent but will not return today. What should be done now?

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Correct answer: D — Start plasma exchange plus glucocorticoids and consider caplacizumab

This is presumptive immune TTP and requires immediate plasma exchange plus glucocorticoids; caplacizumab is used early where available. Platelets can worsen thrombosis unless bleeding is life-threatening, and waiting for ADAMTS13 or treating as DIC causes dangerous delay.

Reference: International Society on Thrombosis and Haemostasis guideline for immune TTP: https://ashpublications.org/bloodadvances/article/4/2/213/428923/452957/ISTH-guidelines-for-treatment-of-thrombotic