Dr Viren Vaghasiya, Dr Divya Bambhaniya, Dr Bhoomika Rupavatiya, Dr Jitendra Nasit,
Volume 19, Issue 6 (11-2025)
Abstract
Background: Sickle cell disease, a hemoglobinopathy due to a point mutation, has a heterogeneous clinical course. HbF level within erythrocytes is believed to be the most important parameter for the severity of disease.
Purpose: To determine whether HbF level, F cell count, and sickle cell percentage after in vitro induction of sickling can predict the severity of the disease.
Methods: All required data was obtained from clinical history, biochemistry, and pathology lab investigations. Statistical analyses were done by using the correlation coefficient and chi-square test to find a significant difference between two variables. Statistical analysis was done using MedCalc software.
Results: Most of the patients are in the mild severity score category; there is 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 were associated with HbF level, F cell count, or the percentage of sickled cells after in vitro induction of sickling. However, a high percentage of F cells was associated with high MCV, MCH, and MCHC and low RDW, LDH, and indirect bilirubin.
Conclusion: This sickle cell disease severity is related to susceptibility of RBCs to hemolysis as indicated by s. LDH and indirect bilirubin levels. However extent of hemolysis may be dependent on multiple factors rather than F cell count or Hb F level only.
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