Abstract

INTRODUCTION: Liver transplantation in patients with Sickle Cell Disease (SCD) is fairly unique; a review article from 2013 noted only 15 reported cases within the literature. This case highlights a patient with SCD who underwent orthotopic liver transplantation. His course was complicated by iron overload due to recurrent exchange transfusions, as well as biopsy-proven acute cellular rejection (ACR), that was inadequately treated with corticosteroids, with no recurrence of rejection once the patient was switched to irradiated red cell exchange transfusions. CASE DESCRIPTION/METHODS: A 32-year-old male with HbSS disease, who had also undergone orthotopic liver transplantation at the age of 26 due to complications of Caroli’s disease presented with persistent abnormal liver tests over the course of several weeks. Graft function was normal during post-transplant until monthly exchange transfusions were initiated to manage his sickle cell crises. During this period he had two episodes of ACR and was noted to have persistent liver test abnormalities despite adequate therapeutic levels of tacrolimus. The patient was switched to receiving irradiated packed red blood cells for his subsequent exchange transfusions, which led to improvement of his liver tests and resolution of his acute cellular rejection. DISCUSSION: Only a limited number of patients with sickle cell disease have undergone liver transplantation, and this is the first case of using irradiated packed red cells for transfusion to help resolve acute cellular rejection. Although it is not thoroughly understood how blood transfusions could induce acute cellular rejection, it may be related to passenger donor leukocytes within the donor blood products triggering an immune response within the transplanted liver. Given our patient’s exposure to higher volumes of transfusions, there may have been enough donor lymphocytes to trigger this response. The use of irradiated blood products is common place in patients that have received hematopoietic stem cell transplants. Current recommendations do not standardize use of irradiated red cells for solid organ transplant (SOT) recipients. Our patient presents an unusual indication for use of irradiated red cells in a SOT patient.

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