Successful liver transplantation for acute sickle cell intrahepatic cholestasis: A case report and review of the literature

Sickle Cell Intrahepatic Cholestasis: Extremely Rare but Fatal Complication of Sickle Cell Disease

“…Acute intrahepatic cholestasis is a fatal condition with supportive treatment as the mainstay of management [ 16 ]. Exchange transfusion is the main and first-line treatment, with a target to decrease the sickle cell burden, decrease the hemoglobin S fraction, and correct the underlying anemia [ 17 , 18 ]. They also reduce viscosity and interrupt the cycle of vaso-occlusion, stasis, and venous thrombosis in the microcirculation [ 18 ].…”

“…They also reduce viscosity and interrupt the cycle of vaso-occlusion, stasis, and venous thrombosis in the microcirculation [ 18 ]. The target Hb S level is below 20%, with exchange transfusions for successful treatment of these episodes [ 14 , 17 , 18 ].…”

“…Patients showing evidence of coagulopathy are treated with fresh frozen plasma [ 17 ]. Temporary renal replacement therapy may be required for acute kidney injury, which may or may not improve with the improvement of liver function [ 14 , 15 ].…”

“…Alkhayyat et al describe liver transplantation as a treatment option if supportive measures fail in two case series describing liver transplantation for patients suffering from liver dysfunction from sickle cell intrahepatic cholestasis [ 17 ]. The cases are from the pediatric population, with the first series describing six patients with one-, five-, and 10-year survival rates of 83.3%, 44.4%, and 44.4%, respectively.…”

“…The cases are from the pediatric population, with the first series describing six patients with one-, five-, and 10-year survival rates of 83.3%, 44.4%, and 44.4%, respectively. The second series described three pediatric patients with a reported survival rate of 66% [ 17 ]. Liver transplant is usually considered as a last resort, and there is significant periprocedural mortality and morbidity in these cases.…”