AJHI2201 - CME/CMLE - The Histopathologic Features of Sickle Cell Hepatopathy: A Multi-Institutional Study-The Histopathologic Features of Sickle Cell Hepatopathy: A Multi-Institutional Study

The Histopathologic Features of Sickle Cell Hepatopathy: A Multi-Institutional Study

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The study assessed liver biopsies from sickle cell disease (SCD) patients to update knowledge on hepatic histopathology and evaluate the utility of liver biopsy. Conducted across four medical institutes, the study included 39 liver biopsies from patients with an average age of 27 years, predominantly female. Most had the hemoglobin SS genotype with severe liver function abnormalities and hepatomegaly being common biopsy indications.<br /><br />The predominant histopathologic features were Kupffer cell erythrophagocytosis (76.9%), hemosiderosis (74.4%), sinusoidal dilatation (71.8%), and intrasinusoidal sickled red cells (69.3%), with mild inflammation and bile duct injury occurring minimally. Advanced fibrosis was present in 28.2% of cases, fewer than historical data suggest.<br /><br />The study found differences from prior reports, noting decreased necrosis and cirrhosis rates, likely due to improved management of SCD. The main histopathologic patterns have evolved, reflecting better disease control and longer life spans due to advances in early diagnosis and treatment.<br /><br />Based on these findings, pathologists should recognize that significant inflammation and bile duct injury are atypical and suggest other etiologies. Liver biopsy should not be routine for suspected SCD-related liver disease due to potential complications but is useful for excluding other causes or assessing fibrosis when non-invasive tests are unreliable.<br /><br />The study underscores the characteristic hepatic changes in SCD and highlights the reduced frequency of severe damage thanks to improved clinical management, advocating cautious use of liver biopsy primarily for differential diagnosis in complex cases.

Keywords

sickle cell disease

liver biopsy

hepatic histopathology

Kupffer cell erythrophagocytosis

hemosiderosis

sinusoidal dilatation

intrasinusoidal sickled red cells

fibrosis

clinical management

differential diagnosis