Background: Sickle cell disease is a hemoglobinopathy caused by a point mutation and has a heterogeneous clinical course. The level of Hb F within erythrocytes is believed to be the most important parameter determining disease severity. The aim of this study was to investigate whether Hb F level, F-cell count, and sickle cell percentage after in vitro induction of sickling can predict the severity of the disease.
Methods: All necessary data were collected from clinical history, biochemistry, and pathology laboratory tests. This was a cross-sectional study with 31 participants. Statistical analyses were performed using the correlation coefficient and chi-square test to identify significant differences between variables. Statistical analysis was performed using MedCalc software.
Results: The majority of patients fell into the mild severity score category, with a lack of severe disease phenotypes. The number of painful episodes, hospitalizations, and cumulative disease severity scores were associated with high levels of LDH and indirect bilirubin. However, none of the clinical disease severity parameters or the overall cumulative disease severity score was associated with Hb F level, F-cell count, or the percentage of sickled cells after in vitro induction of sickling. A high percentage of F-cells was associated with high MCV, MCH, and MCHC and low RDW, LDH, and indirect bilirubin levels.
Conclusion: Sickle cell disease severity is related to the susceptibility of RBCs to hemolysis, as indicated by serum LDH and indirect bilirubin levels. However, the extent of hemolysis may depend on multiple factors rather than F-cell count or Hb F level alone.
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