Deficiency of kappa- or lambda-type immunoglobulins.
A marked imbalance of the two light chain types of immunoglobulins was observed in two young male adults suffering from primary hypogammaglobulinemia and intrinsic factor-deficient pernicious anemia. In one patient, the kappa/lambda light chain ratio of serum immunoglobulins was 0.01; in the other, it was approximately 6 (normal value, 1.8 +/- 0.3). This light chain imbalance was found within each of the three main immunoglobulin classes. The number of mature immunoglobulin-producing cells in the bone marrow and in the mucosa of the gastrointestinal tract was reduced. The relative frequency of kappa- and lambda-producing cells in these tissues corresponded to the kappa/lambda ratio of serum immunoglobulins. However, the number of peripheral lymphocytes with membrane-associated immunoglobulins as well as the percentage distribution of blood lymphocytes with kappa- or lambda-type immunoglobulins on the membrane were within normal limits in both cases. The results suggested a hitherto unknown defect in the maturation of B-cells leading to an abnormal ratio of kappa- and lambda-type immunoglobulin-secreting cells.
D000328:Adult; D000752:Anemia, Pernicious / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D002454:Cell Differentiation; D004406:Dysgammaglobulinemia / Q000276:immunology*; D006801:Humans; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D008214:Lymphocytes / Q000276:immunology; D008297:Male
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains