Case922 -- A Young Boy with Liver Transplantation

Contributed by Bryan Stevens, MD and Alesia Kaplan, MD


The patient is a young boy, less than 2 years of age, with end-stage liver disease secondary to glycogen storage disease, type IV, treated with liver transplantation. The liver segment allograft came from a living closely-related donor.

The patient was transferred to the institution from an outside hospital for liver transplant evaluation. The time between admission and transplantation was complicated by aspiration pneumonia, septic shock, and multi-organ system failure requiring continuous renal replacement therapy and plasma exchange. A close relative elected to be a living related donor for his liver transplantation. The patient's and relative's ABO testing are displayed below.

Use of an ABO minor-mismatch allograft was deemed acceptable in light of the patient's poor clinical condition. During the procedure, the patient received 3 liters of fluid, including packed red blood cells, and lost an estimated 350 mL of blood. The patient did well during the postoperative period. However, his hemoglobin remained persistently low requiring several blood transfusions.

On post-op day #2, the patient had a hemoglobin of 7 g/dL and received 50 mL of type-O pRBCs at 10 mL/kg/hr resulting in an adequate response with hemoglobin of 8.4 g/dL. An evening hemoglobin was found to be 6.8 g/dL and the patient again received type-O pRBCs, resulting in a subsequent hemoglobin of 8.7 g/dL. Additional labs from the same day are included in the table below.

A repeat ABO typing prior to transfusion was performed on the patient during the post-transplant period.

What is the possible diagnosis that would explain ABO reverse typing (see table above)?


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