Three weeks after allogeneic hematopoietic stem cell transplantation with busulfan-based conditioning, a patient develops painful hepatomegaly, weight gain, and hyperbilirubinemia. Liver biopsy shows narrowing of terminal hepatic venules by subendothelial edema and congestion of surrounding sinusoids. What is the underlying injury?

• A Granulomatous inflammation of portal vein radicals
• B Toxic damage to hepatic sinusoidal endothelium with downstream venular occlusion ✓
• C Autoimmune destruction of small intrahepatic bile ducts
• D Thrombosis of the main hepatic veins at their inferior vena caval confluence

Explanation

Sinusoidal obstruction syndrome, formerly called veno-occlusive disease, begins with toxic injury to sinusoidal endothelial cells from high dose chemotherapy or irradiation used in transplant conditioning. Sloughed endothelium obstructs sinusoids and terminal hepatic venules, producing the triad of tender hepatomegaly, fluid retention, and jaundice. Herbal sources such as pyrrolizidine alkaloid containing plants cause the same lesion. Large hepatic vein thrombosis defines Budd-Chiari syndrome, a separate entity.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

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Written and medically reviewed by the StethoPrep medical team.