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

Abstract

Objectives Recent data on hepatic histopathology in patients with sickle cell disease (SCD) are lacking. Methods A total of 39 liver biopsies from SCD patients from 4 medical institutes were systematically evaluated. Results The average age of patients was 27 years; 23 were female. The majority of the patients had hemoglobin SS (33), 3 had hemoglobin SC, and 3 sickle cell trait. Elevated liver functional tests and evaluation for cirrhosis were the main indications for biopsy. At the time of biopsy, most had elevated liver transaminases or hepatomegaly. The most common histopathologic abnormalities were Kupffer cell erythrophagocytosis (76.9%), hemosiderosis (74.4%), sinusoidal dilatation (71.8%), and intrasinusoidal sickled red cells (69.3%). Portal inflammation, lobular inflammation, and bile duct injury were mild to minimal and present in a minority of cases. Advanced fibrosis was present in 28.2% of the cases. Conclusions The typical histopathologic features seen in patients with SCD include Kupffer cell erythrophagocytosis, hemosiderosis, sinusoidal dilatation, and intrasinusoidal sickled red cells in a pauci-inflammatory or uninflamed background. Necrosis is less common than reported in older literature. Pathologists should be aware that significant portal and lobular inflammation, interface activity, and bile duct injury are unusual and may be suggestive of other etiologies.

Congestive Hepatopathy: A Case of Fontan-Associated Liver Disease and Review of Literature

“…Acute hepatic crises in sickle cell anemia patients, presenting with fever, right upper quadrant abdominal pain, and jaundice, are thought to result from sickled erythrocytes obstructing sinusoidal spaces 46 . Liver biopsy is uncommonly performed in this scenario, but features of congestive hepatopathy may be accompanied by dysmorphic red blood cell aggregates in the sinusoidal spaces, hemosiderin deposition, and Kupffer cell erythrophagocytosis 47,48 . In severe cases, centrizonal necrosis may occur.…”

“…46 Liver biopsy is uncommonly performed in this scenario, but features of congestive hepatopathy may be accompanied by dysmorphic red blood cell aggregates in the sinusoidal spaces, hemosiderin deposition, and Kupffer cell erythrophagocytosis. 47,48 In severe cases, centrizonal necrosis may occur. Other etiologies of cellular sinusoidal infiltrates, such as extramedullary hematopoiesis and Epstein-Barr virus infection, may also exhibit histologic features related to sinusoidal blood flow obstruction, such as sinusoidal dilation and congestion.…”

Beta Thalassemia Presenting with Neonatal Cholestasis and Extensive Hemosiderosis: A Case Report

“…However, reports on neonatal cases and natural history are scarce 13) . The histological characteristics of SCH are characterized by Cooper cell erythrocytosis, hemoside rosis, sinusoidal dilatation, and intrasinusoidal sickling 14) . In this case, liver pathology suggested hemosiderin accumulation (siderosis) on two examinations, progression of extramedullary hematopoiesis, perisinusoidal fibrosis, and giant cell formation.…”