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Budd-Chiari syndrome — ABIM Board MCQ

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HardGastroenterology/HepatologyBudd-Chiari syndromeABIM Board

A 58-year-old man has fatigue, infections and bruising. Hemoglobin is 7.4 g/dL, absolute neutrophil count 320/µL, platelets 14,000/µL and reticulocytes 18,000/µL. Bone marrow is markedly hypocellular without dysplasia or malignant infiltration. PNH clone testing is negative, and no HLA-matched sibling donor is available. Which initial disease-directed regimen is most appropriate?

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Correct answer: A — Give horse antithymocyte globulin, cyclosporine and eltrombopag

The best answer is “Give horse antithymocyte globulin, cyclosporine and eltrombopag”. This patient meets criteria for severe acquired aplastic anemia and is older than the group routinely directed to matched-sibling transplantation; no matched sibling is available. Standard immunosuppressive treatment uses horse antithymocyte globulin plus cyclosporine, with eltrombopag added to improve trilineage response. Single-agent thrombopoietin stimulation, incomplete immunosuppression and cytotoxic leukemia therapy are not equivalent frontline regimens.

Reference: ASH 2026 Guideline: Severe Acquired Aplastic Anemia: https://www.hematology.org/education/clinicians/guidelines-and-quality-care/clinical-practice-guidelines/aplastic-anemia