BACKGROUND: Hemolysis due to D incompatibility in the setting of liver transplantation is less frequent than that associated with ABO incompatibility, but can represent an equally adverse event. Approximately 10 percent of ABO-compatible liver transplants involve a D- donor and a D+ recipient.

CASE REPORT: A case of severe D incompatibility resulting from liver transplantation in a 50-year-old O Rh+ man with end-stage liver disease who received an O Rh- liver allograft is reported. A declining hemoglobin level complicated the patient's postoperative course with laboratory evidence of anti-D-mediated hemolysis. Investigations revealed that the transplanted liver was from a female O Rh- donor with detectable antibodies against D, C, and K. The severity of the hemolytic anemia was such that the patient required two separate red blood cell (RBC) exchanges and intermittent RBC transfusions over the course of almost a year. In addition to the use of RBCs negative for D, C, and K, the patient underwent a variety of B-cell suppressive therapies including glucocorticosteroids, mycophenolate mofetil, and rituximab. A normalization of hemoglobin levels and a decrease in serum bilirubin did not occur until after a splenectomy on postoperative Day 321.

CONCLUSION: This represents the sixth and most severe case reported of hemolysis resulting from D incompatibility in liver transplantation. When unexpected serologic findings are identified in a transplant recipient, obtaining more information on the donor may help guide transfusion support.