Therapeutic plasma exchange as a bridge to successful liver transplantation in an adolescent with sickle hepatopathy.

BACKGROUND

Intrahepatic cholestasis, a severe form of sickle cell disease (SCD)-related hepatopathy, is a rare complication of the disease, but with a continued high mortality rate. Although red cell exchange (RCE) is the first-line intervention to manage individuals with sickle hepatopathy, RCE is often not enough to prevent or reverse multiorgan dysfunction (MODS), even in those with hemoglobin S (HbS) suppression below 30%.

CASE REPORT: An 18-year-old with sickle cell disease (HbSS genotype) presented with acute-on-chronic cholestasis secondary to sickle hepatopathy for liver transplant evaluation. However, due to improvement in his liver function after the implementation of a strict every four-week RCE program, he was delisted and instead managed successfully for 5 years on RCE. At age 24 years, he again developed MODS with decompensated acute liver failure and anuric acute renal failure, despite RCE and HbS <30%. He then underwent therapeutic plasma exchange (TPE) with 1:1 fresh frozen plasma/albumin replacement (one plasma volume) with rapid improvement in both renal and synthetic liver function. He continued scheduled TPE as a bridge to successful deceased donor liver transplantation.

CONCLUSION

Our case provides additional evidence of the efficacy of TPE after RCE as a temporizing measure to definitive therapy in the setting of MODS secondary to SCD-related intrahepatic cholestasis. In addition, we show that liver transplantation for intrahepatic cholestasis, a disease with a historically high mortality rate, can be completed safely in patients with SCD.