BIOMAT Database Logo BIOMAT Database Logo

Activity of the alternative complement pathway after splenectomy: comparison to activity in sickle cell disease and hypogammaglobulinemia. 1979

J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston

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.

UI MeSH Term Description Entries
D007136 Immunoglobulins Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses. Globulins, Immune,Immune Globulin,Immune Globulins,Immunoglobulin,Globulin, Immune
D007158 Immunologic Techniques Techniques used to demonstrate or measure an immune response, and to identify or measure antigens using antibodies. Antibody Dissociation,Immunologic Technic,Immunologic Technics,Immunologic Technique,Immunological Technics,Immunological Techniques,Technic, Immunologic,Technics, Immunologic,Technique, Immunologic,Techniques, Immunologic,Antibody Dissociations,Dissociation, Antibody,Dissociations, Antibody,Immunological Technic,Immunological Technique,Technic, Immunological,Technics, Immunological,Technique, Immunological,Techniques, Immunological
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011696 Purpura, Thrombocytopenic Any form of purpura in which the PLATELET COUNT is decreased. Many forms are thought to be caused by immunological mechanisms. Purpura, Thrombopenic,Purpuras, Thrombocytopenic,Purpuras, Thrombopenic,Thrombocytopenic Purpura,Thrombocytopenic Purpuras,Thrombopenic Purpura,Thrombopenic Purpuras
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003167 Complement Activation The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES. Activation, Complement,Activations, Complement,Complement Activations
D003170 Complement Pathway, Alternative Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX. Alternative Complement Pathway,Properdin Pathway,Alternative Complement Activation Pathway,Complement Activation Pathway, Alternative

Related Publications

J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Alternative pathway of complement in sickle cell disease.
April 1977, Pediatric research,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Alternative complement pathway activity in sera from patients with sickle cell disease.
January 1976, Clinical and experimental immunology,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Increased activation of the alternative complement pathway in sickle cell disease.
October 1985, Clinical immunology and immunopathology,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Contribution of alternative complement pathway to delayed hemolytic transfusion reaction in sickle cell disease.
October 2018, Haematologica,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Splenectomy in sickle cell disease.
September 1999, The Journal of the Association of Physicians of India,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Splenectomy in sickle-cell disease.
August 1979, Annals of internal medicine,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Activation of the alternative complement pathway by red blood cells from patients with sickle cell disease.
May 1994, Clinical immunology and immunopathology,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
[Partial splenectomy in sickle cell disease].
April 2015, Anales de pediatria (Barcelona, Spain : 2003),
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Activation of the alternative complement pathway by exposure of phosphatidylethanolamine and phosphatidylserine on erythrocytes from sickle cell disease patients.
September 1993, The Journal of clinical investigation,
J M Corry, and R B Polhill, and S R Edmonds, and R B Johnston
Alternative pathway activation in sickle cell disease and beta-thalassemia major.
July 1978, Journal of the National Medical Association,
Copied contents to your clipboard!