Activity of the alternative complement pathway after splenectomy: comparison to activity in sickle cell disease and hypogammaglobulinemia.
Patients with sickle cell disease and individuals who have undergone splenectomy share defects of certain host defense mechanisms and a predisposition to severe pyogenic bacterial infections. Since patients with sickle cell disease can have deficient activity of the alternative complement pathway, we have tested such activity in sera from splenectomized children and adults. A new kinetic hemolytic assay has been used, and we have compared results to those obtained with sera from patients with sickle cell disease or hypogammaglobulinemia. Sera from six of 58 splenectomized individuals (10%) had defective function of the alternative pathway, compared to 10 of 62 sera from patients with sickle cell disease (16%) and 10 of 18 sera from hypogammaglobulinemic patients (56%). Deficiency of antibody, a rate-influencing component of alternative pathway activity in this system, appears responsible for deficient activity in the hypogammaglobulinemic sera. The molecular basis for the deficiency found in sickle cell disease or after splenectomy is not clear. Defective function of the alternative complement pathway could contribute to the increased predisposition to bacterial infection that exists in these three patient groups.
D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000276:immunology* / Q000628:therapy; D000755:Anemia, Sickle Cell / Q000276:immunology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003167:Complement Activation; D003176:Complement C3 / Q000032:analysis; D003170:Complement Pathway, Alternative; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007158:Immunologic Techniques; D007223:Infant; D008297:Male; D008875:Middle Aged; D011696:Purpura, Thrombocytopenic / Q000276:immunology; D013103:Spherocytosis, Hereditary / Q000276:immunology; D013156:Splenectomy
D003176:Complement C3; D007136:Immunoglobulins