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Immune thrombotic thrombocytopenic purpura — ABIM Board MCQ

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ModerateHematology/OncologyImmune thrombotic thrombocytopenic purpuraABIM Board

A 38-year-old woman has confusion, petechiae, and acute kidney injury. Hemoglobin is 7.8 g/dL, platelets are 14,000/mm3, creatinine is 1.7 mg/dL, LDH is elevated, haptoglobin is undetectable, and smear shows schistocytes. PT and aPTT are normal. ADAMTS13 testing is sent. Which of the following is the most appropriate management?

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Correct answer: DImmediate plasma exchange plus glucocorticoids

Thrombotic thrombocytopenic purpura is a medical emergency with microangiopathic hemolytic anemia, thrombocytopenia, neurologic findings, and relatively normal coagulation tests. Plasma exchange and immunosuppression should start immediately without waiting for ADAMTS13 results.

Reference: ASH Thrombotic Microangiopathy/TTP Guidance