IMMUNE DYSFUNCTION IN PATIENTS WITH SICKLE CELL ANEMIA

Abstract

Background: Sickle cell anemia (SCA) is a hemoglobinopathic disease associated with a chronic
hemolysis and vasoocclusive events. Patients with sickle cell anemia (SCA) have increased
susceptibility to impairment of immune function. The aim of the present study to evaluate immune
dysfunction in patient with sickle cell anemia. Patients and methods: A cross-sectional study
Included pediatric patients with SCD who were following up regularly, at Pediatric Hematology
Outpatient Cinics, Pediatric Department in Zagazig University Hospitals. All patients were subjected to
complete history taking with special emphasis. Clinical and special investigation including T-cell
function evaluation, CD3, CD4, CD8, CD19 and CD56 were performed using immune phenotype
analysis.
Results: There is statistically significant positive correlation between hemoglobin S and interleukin 6.
There is statistically significant negative correlation between hemoglobin S and both CD 4 and, CD 56
and IgG. There is statistically non-significant correlation between hemoglobin S and either CD3, CD8,
CD19, IgM, IgE, and IgA. There is statistically significant association between vasocclusive crisis and
all of CD4, CD8, CD 19 and CD 56, and IgA. All were significantly lower in patients in vasocclusive
crisis. There is statistically non-significant association between vasocclusive crisis and CD 3. There is
statistically significant association between vasocclusive crisis and all of IgM, IgG, IgE, and IgA.
There is statistically significant association between vasocclusive crisis and all of IL-6. It was
significantly higher in patients in vasocclusive crisis.
Conclusion: It can be concluded that sickle cell anemia (SCA) patients have increased susceptibility to
impairment of immune function. A significant association was reported between vaso-occlusive crisis
and all of IgM, IgG, IgE, and IgA. All were significantly higher in patients in vaso-occlusive crisis. In
addition; IL-6 was significantly higher in patients in vasocclusive crisis.