Hepatic Enzyme Abnormalities and Their Association With Hematological Parameters in Sickle Cell Disease: A...

The polymerization of deoxygenated hemoglobin S, resulting from a genetic mutation in sickle cell disease (SCD), leads to damage in multiple organs, including renal, cardiopulmonary, and cerebrovascular systems. The liver is also commonly affected, resulting in "sickle cell liver disease," which may lead to progressive fibrosis and impaired liver function in adulthood. We hypothesized that hepatic enzyme abnormalities are frequent in patients with sickle cell disease and are associated with underlying hematological alterations related to hemolysis. This study aimed to determine the frequency of hepatic enzyme abnormalities in patients with sickle cell disease and to examine their association with hematological parameters.

A 4-month case-control study was conducted at the Hematology Department of the Bafoussam Regional Hospital. Following informed consent, blood samples were collected in EDTA tubes for complete blood counts (performed by flow cytometry) and in plain tubes for biochemical analyses. These included the enzymatic activities of alkaline phosphatase (ALP), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyl transferase (GGT), measured using kinetic spectrophotometric methods, as well as albumin concentration, determined using a colorimetric spectrophotometric method. Based on these biochemical parameters, patterns of hepatocellular injury, cholestasis, and mixed liver injury were identified. Data were analyzed using R software version 4.1.1.

A total of 167 participants were enrolled, including 94 patients with homozygous sickle cell disease (SS) and 73 healthy controls (AA). ALT levels were significantly higher in patients than in controls, with median values of 18.2 U/L [14.0-26.7] versus 15.1 U/L [12.0-24.0] (p = 0.007). AST levels were also significantly elevated in patients, with 68 (72.3%) showing increased values compared to none among controls (p < 0.001). Similarly, ALP levels were higher in patients, with 60 (63.8%) presenting elevated values compared to none in controls (p < 0.001). GGT levels were significantly increased in patients compared to controls, with median values of 42.0 U/L [35.0-52.2] versus 27.0 U/L [24.0-30.0] (p < 0.001). Albumin levels were significantly lower in patients, with 36 (38.3%) exhibiting hypoalbuminemia compared to none in controls (p < 0.001). Furthermore, biochemical suspicion of cholestatic injury was significantly more frequent in patients (23.4%) than in controls (0%) (p < 0.001), while 8 (8.5%) patients showed biochemical suspicion of hepatocellular injury compared to none in controls (p = 0.01).

These findings indicate significant liver involvement in patients with sickle cell disease, characterized by hepatocellular injury, cholestasis, and impaired synthetic function. Regular biochemical monitoring of liver function is therefore essential to improve disease management and prevent complications.