Acute Liver Failure in Sickle Cell Disease: A Perfect Storm

Abstract

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.

Acute-on-Chronic Liver Failure From Acquired Hemochromatosis in a Patient With Sickle Cell Disease

“…In addition to chronic disease, patients with SCD can have acute exacerbations of their chronic problems. There are a few known acute sickle cell hepatopathies that are present in a spectrum of diseases, including acute sickle cell hepatic crisis, acute hepatic sequestration, and acute intrahepatic cholestasis [5][6].…”

Clinical, Hematological, Biochemical and Radiological Characteristics for Patients With Splenic Infarction: Case Series With Literature Review

“…Hereditary spherocytosis [52] Sickle cell disease [53][54][55][56][57] Hemoglobin SE disease [58] β-Thalassemia [59] Protein C deficiency [60] Antiphospholipid syndrome [61] Vasculitis ANCA-associated vasculitis [62][63][64] Eosinophilic granulomatosis with polyangiitis [65] Cardiovascular disease, atrial fibrillation Atrial fibrillation [66,67] COVID-19: coronavirus disease 2019; ANCA: anti-neutrophil cytoplasmic antibody. rate (30.7%) was found in patients with infective endocarditis [4].…”