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Alternative pathway of complement in sickle cell disease

R G Strauss, T Asbrock, J Forristal

Pediatric Research

|April 1, 1977

Summary

This study found no complement abnormalities in pediatric sickle cell disease (SCD) patients. Complement component levels and alternative pathway function were comparable to healthy controls, suggesting SCD does not inherently impair complement activity.

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Area of Science:

Immunology
Hematology
Pediatrics

Background:

Sickle cell disease (SCD) is a genetic blood disorder.
Complement system plays a crucial role in immune defense.
Previous studies suggested potential complement involvement in SCD complications.

Purpose of the Study:

To investigate complement system abnormalities in pediatric patients with sickle cell disease.
To assess the function of the alternative pathway of complement activation in SCD.
To determine if complement deficiencies contribute to SCD pathogenesis.

Main Methods:

Sera from 31 pediatric SCD patients (10 months-20 years) and controls were analyzed.
Concentrations of C3, factor B, CH50, properdin, and C3b inactivator were measured.
Alternative pathway activation was assessed using inulin and cobra venom factor, measuring factor B activation, C3 cleavage, and hemolytic activity.

Main Results:

No significant differences in C3, factor B, CH50, properdin, or C3b inactivator levels were observed between SCD patients and controls.
Alternative pathway activation by inulin and cobra venom factor showed comparable function in SCD sera versus control sera.
Slightly low C3b inactivator in a few patients did not appear functionally significant.

Conclusions:

The study did not identify complement abnormalities in pediatric patients with sickle cell disease using the employed methods.
The alternative pathway of complement appears to function normally in SCD patients.
Inulin is not a suitable activator for assessing alternative pathway function in SCD sera.

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