Regional deficiency of secretory IgA in a patient with combined immunodeficiency of the ADA deficient type.
The IgA system in a patient with SCID and ADA deficiency showed heterogeneity. Serum IgA and stool secretory IgA (SIgA) levels were normal, but with altered kappa/lambda and A1/A2 subclass ratios; IgA in saliva and urine was deficient. Amounts of secretory component were normal. Jejunal and rectal biopsies showed prominent lymphonodular hyperplasia, but no cells containing IgA. A normal serum IgA level therefore does not always predict an intact secretory IgA system.
D000243:Adenosine Deaminase / Q000172:deficiency*; D000367:Age Factors; D002675:Child, Preschool; D005243:Feces / Q000032:analysis; D005260:Female; D006801:Humans; D006965:Hyperplasia; D007070:Immunoglobulin A / Q000032:analysis; D007071:Immunoglobulin A, Secretory / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D009700:Nucleoside Deaminases / Q000172:deficiency*; D011863:Radioimmunoassay; D012463:Saliva / Q000276:immunology
D007070:Immunoglobulin A; D007071:Immunoglobulin A, Secretory; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase