Increased susceptibility to infection in sickle cell disease: defects of opsonization and of splenic function.
Patients with sickle cell disease (SCD) have an increased susceptibility to bacterial infections and die more frequently from infection than any other cause. Their sera do not promote the phagocytosis of pneumococci normally, apparently because of an inability to utilize the alternate pathway for C3 fixation as a means of opsonization. The splenic dysfunction which exists in SCD may result in depressed synthesis of alternate pathway factor(s), as well as in decreased phagocytic clearance of bacteria.
D000755:Anemia, Sickle Cell / Q000150:complications* / Q000276:immunology; D000906:Antibodies; D000907:Antibodies, Bacterial; D000917:Antibody Formation; D001424:Bacterial Infections / Q000209:etiology*; D001665:Binding Sites; D002462:Cell Membrane / Q000276:immunology; D003176:Complement C3; D003165:Complement System Proteins / Q000378:metabolism; D004533:Egtazic Acid; D005838:Genotype; D006451:Hemoglobin, Sickle; D006801:Humans; D007962:Leukocytes / Q000276:immunology / Q000378:metabolism; D008581:Meningitis / Q000209:etiology; D009895:Opsonin Proteins / Q000032:analysis; D010587:Phagocytosis; D011414:Properdin / Q000378:metabolism; D013154:Spleen / Q000276:immunology*; D013296:Streptococcus pneumoniae / Q000276:immunology; D015054:Zymosan
D000906:Antibodies; D000907:Antibodies, Bacterial; D003176:Complement C3; D006451:Hemoglobin, Sickle; D009895:Opsonin Proteins; D011414:Properdin; D004533:Egtazic Acid; D003165:Complement System Proteins; D015054:Zymosan