Immunological Studies in Sickle Cell Disease: Comparison of Homozygote Mild and Severe Variants

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Çetiner S. , Akoğlu T., Kılınç Y. , Akoğlu E., Kümi M.

CLINICAL IMMUNOLOGY AND IMMUNOPATHOLOGY, vol.53, pp.32-39, 1989 (Journal Indexed in SCI Expanded)

  • Publication Type: Article / Article
  • Volume: 53
  • Publication Date: 1989
  • Page Numbers: pp.32-39


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. Cl 1989 Academtc Press. Inc.