Budd-Chiari syndrome recurring in a transplanted liver

Abstract

A patient with Budd-Chiari syndrome who underwent orthotopic liver transplantation and developed recurrent disease is described. The immediate postoperative period was complicated by multiple thrombotic episodes, followed by a period of apparent remission associated with the initiation of coumadin and persantine therapy. After discontinuation of such antithrombotic therapy in order to biopsy the liver, the patient experienced another series of clinically overt vascular thromboses and ultimately died of sepsis 15 mo posttransplantation after a prolonged and complicated terminal hospital course. At autopsy, recurrent Budd-Chiari syndrome as well as thromboses in numerous other organs was demonstrated.

Figure 1

Original liver with dilated and congested sinusoids. The two-cell-thick plates indicate regeneration. Note partial “occlusion of radicle of terminal hepatic vein by organizing thrombosis. (H & E; ×93.)

Figure 2

Transplant liver with occlusion of terminal hepatic vein due to organizing thrombosis with partial recanalization. Area of zonal necrosis is indicated by arrows. (H & E; ×40; inset, ×93.)

Figure 3

Transplant liver with area of zonal necrosis in centrilobular area (Rappaport zone 3) with organizing thrombosis of vein in its center. (Verhoff-VanGieson; ×93.)