• Acute hepatic crisis
• Sickle cell disease
• Sickle cell hepatopathy
• Sickle cell intrahepatic cholestasis

• Humans
• Anemia, Sickle Cell/complications
• Anemia, Sickle Cell/diagnosis
• Cholestasis, Intrahepatic/etiology
• Cholestasis, Intrahepatic/complications
• Abdominal Pain/etiology

• Auto-immune liver diseases
• Cholangiopathy
• Liver
• Liver transplantation
• Porto-sinusoidal disease
• SCD hepatopathy
• Sickle cell disease
• Sinusoidal obstruction syndrome
• Stem cells transplantation

Sickle cell intrahepatic cholestasis (SCIC) is a rare but potentially fatal complication of sickle cell disease (SCD), with high mortality, observed mainly in patients with homozygous sickle cell anemia. Herein, we have reported a case of severe SCIC with a poor outcome despite aggressive measures including exchange transfusion and use of vasopressors.

The patient was admitted with generalized weakness, confusion, rigors, chills, and signs of hepatic failure, such as hyperbilirubinemia, hypoalbuminemia, and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. The patient received two exchange transfusions, but he continued to deteriorate clinically despite exchange transfusion and developed hemorrhagic shock and multiorgan failure. The patient was made comfort care as per family wishes. This case emphasizes the importance of early diagnosis of sickle cell intrahepatic cholestasis and poor prognosis despite aggressive measures.

• Aged
• Anemia, Sickle Cell/complications
• Anemia, Sickle Cell/diagnosis
• Anemia, Sickle Cell/epidemiology
• Carcinoma, Hepatocellular/epidemiology
• Carcinoma, Hepatocellular/pathology
• Carcinoma, Hepatocellular/therapy
• Humans
• Liver Neoplasms/epidemiology
• Liver Neoplasms/pathology
• Liver Neoplasms/therapy
• Medicare
• Propensity Score
• Retrospective Studies
• Treatment Outcome
• United States/epidemiology

• HHSN261201000140C NCI NIH HHS
• HHSN261201000035I NCI NIH HHS
• HHSN261201000034C NCI NIH HHS
• U58 DP003862 NCCDPHP CDC HHS
• K08 CA245220 NCI NIH HHS
• HHSN261201000035C NCI NIH HHS

• Anemia, Sickle Cell/diagnosis
• Anemia, Sickle Cell/surgery
• End Stage Liver Disease
• Humans
• Liver Cirrhosis
• Liver Transplantation/adverse effects

Sickle cell hepatopathy is a well-described but uncommonly seen complication of sickle cell disease and is usually caused by multiple overlapping processes. A more acute liver complication is hepatic sequestration which is important to recognize in order to initiate life-saving treatment. A 33-year-old woman with sickle cell disease complicated by painful crises, splenic infarction and significant alcohol abuse presented with gastrointestinal distress, pain crisis, acute-on-chronic anemia, and hyperbilirubinemia in the setting of greater than baseline alcohol consumption. She was found to have hepatomegaly, encephalopathy, severe jaundice, and severe hyperbilirubinemia. She was treated with red cell exchange and supportive care which resulted in an improvement in her symptoms as well as hyperbilirubinemia. She was discharged with plans for monthly red cell exchange, iron chelation therapy, and close monitoring of liver disease was planned upon discharge. This case illustrates that chronic liver disease can occur in sickle cell disease (Hgb SS) especially in the setting of acquired iron overload. More acutely, sequestration is a serious and life-threatening complication of sickle cell disease that can culminate in acute liver failure. Primary treatment for hepatic sequestration is red cell exchange along with management of contributing comorbidities, and symptomatic management of encephalopathy. In end-stage liver disease, transplantation may be considered in the context of the patient’s clinical status.

• adult sickle cell anemia
• congestive hepatopathy
• liver and gall bladder disease
• liver infarction
• rare liver disease
• sickle cell complications
• sickle cell crisis
• sickle cell disease complications
• sickle cell disease: scd
• sickle cell hbsc

• Anemia, Sickle Cell/complications
• Cholestasis, Intrahepatic/etiology
• Cholestasis, Intrahepatic/therapy
• Humans

• Health disparities
• Liver transplantation
• Scientific registry of transplant recipients
• Sickle cell disease

Sickle cell hepatopathy (SCH) is an inclusive term referring to any liver dysfunction among patients with sickle cell disease. Acute sickle cell intrahepatic cholestasis is one of the rarest and most fatal presentations of SCH. We present the 23rd reported case of liver transplantation (LT) for SCH; a rare case of acute sickle cell intrahepatic cholestasis managed with LT from a hepatitis C virus (HCV) nucleic acid amplification test positive donor.

A 29-year-old male with a past medical history of sickle cell disease presented with vaso-occlusive pain crisis. On examination, he had jaundice and a soft, non-tender abdomen. Initially he was alert and fully oriented; within 24 h he developed new-onset confusion. Laboratory evaluation was notable for hyperbilirubinemia, leukocytosis, anemia, thrombocytopenia, acute kidney injury and elevated international normalized ratio (INR). Imaging by ultrasound and computed tomography scan suggested a cirrhotic liver morphology with no evidence of biliary ductal dilatation. The patient was diagnosed with acute sickle cell intrahepatic cholestasis after excluding competing etiologies of acute liver injury. He underwent LT from an HCV nucleic acid amplification test positive donor 9 d after initial presentation. The liver explant was notable for widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells and cholestasis. On postoperative day 3, HCV RNA was detectable in the patient's peripheral blood and anti-HCV therapy with glecaprevir/pibrentasvir was initiated on postoperative day 23. He subsequently achieved sustained virologic response after completing 3 mo of therapy and has been followed clinically for 12 mo post-transplant.

This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis.

• Case report
• Sickle cell hepatopathy
• Acute intrahepatic cholestasis
• Liver transplant
• Hepatitis C virus
• Post-operative surveillance

Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution.

• Apheresis
• Exchange transfusion
• Red blood cell exchange
• Sickle cell disease
• Sickle cell hepatopathy