Immunological studies in sickle cell disease: comparison of homozygote mild and severe variants.

Cellular and humoral immune functions in patients suffering from severe and mild forms of homozygous sickle cell disease (SCD) were compared with those of healthy control subjects. Random neutrophil migration, chemotactic activity, and lymphocyte transformation index were all defective in individuals with severe variants of SCD when compared with individuals with mild disease or healthy controls. In contrast, serum opsonization activity was significantly reduced in both severe and mild variants of SCD. There were no statistical differences between serum immunoglobulin (IgA, IgG, and IgM) or complement C3 levels in any of the three groups. These results demonstrate that even though individuals with the mild variant of SCD possess two S genes, their immune functions are generally normal and in parallel with their clinical and hematological status. The one area of impaired immune function is their defective serum opsonization activity and this may explain their sensitivity to certain infections.