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Type 2 diabetes with albuminuric chronic kidney disease — ABIM Board MCQ

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HardEndocrinology/MetabolismType 2 diabetes with albuminuric chronic kidney diseaseABIM Board

A 34-year-old woman has confusion, platelets 14,000/µL, schistocytes, undetectable haptoglobin and creatinine 1.4 mg/dL. PT and fibrinogen are normal, and her PLASMIC score indicates high probability of severe ADAMTS13 deficiency. ADAMTS13 testing is pending. Which disease-directed regimen should begin now?

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Correct answer: B — Begin plasma exchange, glucocorticoids and caplacizumab without awaiting ADAMTS13

The best answer is “Begin plasma exchange, glucocorticoids and caplacizumab without awaiting ADAMTS13”. A high-probability clinical presentation of immune TTP is a treatment emergency. Daily therapeutic plasma exchange replaces ADAMTS13 and removes autoantibody, glucocorticoids suppress inhibitor production, and caplacizumab rapidly blocks von Willebrand factor-mediated platelet adhesion. Therapy should not wait for the ADAMTS13 result; platelet transfusion is generally avoided without life-threatening bleeding.

Reference: FDA Approval: Caplacizumab for Acquired TTP: https://www.fda.gov/drugs/news-events-human-drugs/fda-approves-therapy-rare-blood-disorder-pediatric-patients-12-years-and-older