Associate Professor; Associate Medical Director University of California San Diego San Diego, California, United States
Background/Case Studies: A 30-year-old female with sickle cell disease developed severe iron overload from chronic transfusions, resulting in cirrhosis and decompensated liver failure. She was denied liver transplantation by multiple centers due to ongoing sickle crises and was offered hospice care before transferring to our center for liver transplant evaluation.
Study
Design/Methods: The transfusion medicine (TM) service was alerted to the patient’s complex alloimmunization history (Table 1), which was clarified with multiple centers. Approximately 2.5% of the blood donor population was expected to match the patient’s antigen requirements; < 1% would match her full phenotype. The TM service convened a multidisciplinary group with transplant surgery, hepatology, sickle cell/hematology, apheresis, and the blood supplier to discuss the patient’s anticipated blood product needs (for both the transplant and long-term management), feasibility, and risks. To maximize product availability, the team elected to accept units untested for certain antigens and forgo a prophylactic antigen requirement (Table 1), while the blood supplier worked over several days to secure 26 units from across the country. To minimize the patient’s hemoglobin S (HgbS) % prior to surgery and to potentially mitigate further alloimmunization risk, the patient underwent red cell exchange and was administered prophylactic rituximab.
Results/Findings: The patient’s candidacy for transplant was approved. She developed shock and multi-organ failure. She received an offer two days after listing and underwent liver transplantation from a deceased donor the following day. A total of 13 RBC units were employed in the surgery. The patient was extubated the day after transplant, received four simple RBC transfusions in the post-operative period, and underwent red cell exchange before discharge. No new antibodies have been identified, and the patient plans to undergo monthly RBC exchanges for one year post-transplant. Conclusions: Successful, life-saving liver transplantation in a highly alloimmunized patient with sickle cell disease is possible and requires open, collaborative communication among the TM service, clinical specialists, and the blood supplier. Removing select antigen requirements and executing nationwide searches for matching blood were critical to providing adequate perioperative transfusion support in this case.
Importance of research: This case illustrates a successful liver transplantation in a highly alloimmunized patient with SCD. Data on these surgeries in patients with SCD are very limited, and institutions may struggle with obtaining high volumes of phenotype-matched RBCs for other life-saving procedures. Lessons learned from this case aim to address both of these knowledge gaps. Demonstrating the success of this surgery may help promote equitable access to liver transplantation, inclusive of patients with SCD.
