The patient was 52 years old and underwent a liver transplant 13 years after his hematopoietic stem cell transplant (HSCT) to treat his sickle cell disease (SCD).
Liver transplantation can be a viable option for select patients who have undergone a hematopoietic stem cell transplantation (HSCT) to treat sickle cell anemia, according to a new published case report.
The case, from Brazil, is believed to represent the first published instance of a post-HSCT liver transplant leading to long-term favorable results. The report was published in Transplantation Proceedings.
Patients with sickle cell disease (SCD), an inherited red blood cell disorder, face a host of complications, noted the authors.
“Distortion of red blood cells in turn triggers abnormal rheology, abnormal adhesiveness-mediated vaso-occlusion, ischemic tissue damage, and endothelial damage,” they wrote. “The hypercoagulable state, hypoxia, and inflammation generation all cause different damage to organs.”
Many patients with SCD experience iron overload in the liver, owing to causes such as multiple blood transfusions, prolonged ineffective erythropoiesis, hemolysis, and increased iron absorption, the investigators noted. HSCT is considered curative for SCD, but it cannot reverse organ damage, such as liver fibrosis and siderosis, the authors noted
“HSCT recipients often have a long history of transfusion support and commonly develop tissue iron overload, with marked hemosiderosis on liver histopathologic examination and elevated iron concentration on biochemical determination,” they said.
In patients with mild siderosis, HSCT can lead to a decrease in iron stores, but the investigators said with moderate to severe siderosis, the slow depletion of iron stores is insufficient to hold off liver dysfunction.
“For this reason, several transplant teams have developed iron-depletion protocols with satisfactory results,” they wrote.
In the case at the center of this report, a 52-year-old male patient had experienced episodes of priapism since puberty, which continued despite hydroxyurea. The patient underwent allogeneic HSCT with a donation from his brother, and the procedure appeared to go well, with no clinical manifestations of SCD following the transplant. However, the patient had already suffered significant liver fibrosis, and his liver function continued to decline after the HSCT. Twelve years following the HSCT procedure, the patient had jaundice and ascites.
“A few months after these symptoms appeared, he displayed acute decompensation of the liver disease with no identifiable cause, being admitted to the emergency room with a Model for End Stage Liver Disease score of 31, Child-Pugh score of 11 (class C), hemoglobin level of 7 g/dL, aspartate aminotransferase of 64 U/L, alanine aminotransferase of 42 U/L, and total bilirubin level of 46.8 mg/dL,” the authors reported.
The patient was slated to receive a liver transplant, and he underwent the procedure 13 years after his HSCT procedure. The liver transplant was successful, and the patient was put on an immunosuppressive regimen and discharged from the hospital 17 days later.
“The liver explant showed liver cirrhosis associated with intense siderosis in hepatocytes and marked sinusoidal dilatation and congestion, with sickling of red blood cells,” the authors said.
Three years following the liver transplant, the patient had good liver and bone marrow function, no symptoms of SCD, and remained under outpatient observation, they said.
The investigators said the report shows that liver transplant can be a life-saving procedure in certain patients with liver complications despite HSCT.
“In patients with significant hepatic impairment as a result of iron overload before HSCT, progression of liver dysfunction may occur after the procedure,” they wrote. “Liver transplantation is a viable treatment option for patients who develop cirrhosis after HSCT.”
de Sousa Arantes Ferreira G, Ferreira CA, Watanabe ALC, et al. Liver transplantation after hematopoietic stem cell transplant for the treatment of sickle cell disease: a case report. Transplant Proc. Published online June 1, 2022. doi:10.1016/j.transproceed.2022.03.047