Association of fetal hemoglobin level with frequency of acute pain episodes in sickle cell disease (HbS-only phenotype) patients

Abstract Background Sickle cell disease (SCD) is a Mendelian single gene disorder with highly variable phenotypic expression. In the present study, we analyzed the influence of HbF, alpha thalassemia and other hematological indices to determine their association with acute pain episodes. Method This case control study consisted of SCD subjects with HbS phenotype experiencing three or more acute pain episodes in last twelve months (cases) and without any episode of acute pain during last twelve months (controls). Hematological parameters, HbF, and presence of alpha thalassemia were assessed in all subjects. Results A statistically significant difference between HbF levels (P   25% (OR: 0.37, 95% CI: 0.18‐0.77, P  t -test, P  Conclusion Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCD patients.