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The immunological aspects of sickle cell syndrome with particular reference to circulating immune complexes
1Department of Clinical Pathology, School of Clinical Sciences, College of Medicine, University of Lagos, Nigeria.
Insights
Individuals with sickle cell syndrome (SCS) have a poorly understood immune status, increasing infection susceptibility. Elevated IgM levels during painful crises may contribute to vaso-occlusive symptoms.
Area of Science:
- Immunology
- Hematology
Background:
- Sickle cell syndrome (SCS) is associated with increased susceptibility to infections.
- The immunological status of SCS patients is not well understood.
- Immune complex pathology may play a role in SCS complications.
Purpose of the Study:
- To review the immunological aspects of sickle cell syndrome.
- To investigate the immune status of SCS patients.
- To explore the role of immune complexes and complement in SCS.
Main Methods:
- Literature review.
- Quantification of C4 complement protein using laser nephelometry.
- Measurement of serum IgG and IgM levels.
Main Results:
- SCS patients exhibit elevated serum IgG and IgM levels.
- A significant increase in serum IgM was observed during vaso-occlusive painful crises.
- Complement protein consumption correlated positively with immune complex levels.
Conclusions:
- SCS patients are generally susceptible to immune complex pathology.
- Elevated IgM may contribute to the vaso-occlusive painful crisis in SCS.
- Further research is needed to fully elucidate the immune status in SCS.
Abstract:
A concise but critical review of the literature on the immunological aspects of sickle cell syndrome (SCS) was carried out. This exercise revealed our poor understanding of the immune status of these persons, while most of them succumb to overwhelming infections. We used laser nephelometric technique to quantitate the C4. Unlike the serum IgM complex, we found that IgG and IgM were raised in these persons. However, a significant rise in serum IgM was noted during the symptom of vaso-occlusive painful crisis. The profile of complement protein consumption correlated positively with the levels of the immune complexes. We suggest that sickle cell persons are generally more susceptible to immune complex pathology and the possible contribution of IgM to the symptom of vaso-occlusive painful crisis was discussed.