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Neutropenic fever — ABIM Board MCQ

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HardHematology/OncologyNeutropenic feverABIM Board

A 31-year-old woman has confusion, petechiae, hemoglobin 7.8 g/dL, platelets 14,000/microL, undetectable haptoglobin, schistocytes, normal coagulation studies, and creatinine 1.4 mg/dL. An ADAMTS13 activity assay has been sent and will return in four days. What is the best immediate treatment?

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Correct answer: A — Plasma exchange, glucocorticoids, and caplacizumab

The best answer is “Plasma exchange, glucocorticoids, and caplacizumab”. The clinical syndrome and high pretest probability indicate immune TTP, which is fatal if treatment waits for ADAMTS13 confirmation. Plasma exchange and immunosuppression begin immediately, and caplacizumab reduces early microvascular thrombosis while the autoimmune process is controlled. Platelets are avoided unless life-threatening bleeding requires them.

Reference: ASH ISTH Guidelines for Treatment of Thrombotic Thrombocytopenic Purpura: https://pmc.ncbi.nlm.nih.gov/articles/PMC8091490/