Hemolysis scavenger proteins and renal function marker in children with sickle cell disease at steady state: A cross‐sectional study

BACKGROUND AND AIMS: Hemolysis is a fundamental feature of sickle cell disease (SCD) contributing to the vaso‐occlusive crisis of patients. The objectives of the study were to assess the link between hemolysis proteins and hematological parameters, and to validate cystatin C (CYS C) as a potent renal marker in diagnoising SCD. METHOD: Here, a cross‐sectional study carried out at the pediatric SCD clinic of the Komfo Anokye Teaching Hospital comprised 90 SCD children (HbSC, HbSF, and HbSS). ANOVA, t‐test, and Spearman's rank correlation analysis were done. Elevated proteins levels were compared to standard values; alpha‐1 microglobulin (A1M) (1.8−65 µg/L), CYS C (0.1−4.5 µmol/L), and haemopexin (HPX) (500−1500 µg/mL). RESULTS: The mean (standard deviation) age of participants was 9.830 (±0.3217) years, and 46% of them were males. From simple descriptive analysis, we observed that all but one patient had their HPX level below the reference range (<500 µg/mL). Here, A1M levels were shown to be within the appropriate reference range for all the patients except few patients. CYS C levels were also all within the required reference values. A Spearman's rank correlation test between full blood count and HPX generally suggested a weak but positive correlation; RBC (coef. = 0.2448; p = 0.0248), HGB (coef. = 0.2310; p = 0.030), hematocrit (coef. = 0.2509; p = 0.020), and platelet (coef. = 0.1545; p = 0.160). Mean corpuscular volume (coef. = ‐0.5645; p = 0.610) had a stronger but negative correlation with HPX. This study depicts a positive and stronger association between CYS C and HPX levels (coef. = 0.9996; p < 0.0001), validating the use of CYS C as a useful marker of renal function in persons with SCDs. CONCLUSION: In the present study, we show that A1M levels were normal for most of the patients, hence CYS C levels are not alarming in this study. Further, there exists a correlation between hemolysis scavenger proteins and hematological parameters.