title: [Unusual rearrangements of immunoglobulin genes in a patient with IgD/lambda plasmacytoma].
abstract: The mechanisms of Ig gene expression in B-lymphocytes are not yet fully understood. A hierarchy of Ig gene rearrangements has been suggested starting with Ig heavy chain genes followed by kappa and lambda light chain genes. According to this principle, B-cells with productive lambda light chain expression have their kappa genes either unproductively rearranged or deleted. By examining B-cells from patients with various lymphoproliferative diseases the developmental hierarchy of the Ig gene rearrangements could be confirmed with one notable exception: in a patient with IgD/lambda-plasmocytoma we found the kappa genes in germ line configuration. This finding points to an aberrant light chain gene expression in IgD-producing plasma cells.
mesh_terms: D000483:Alleles; D001402:B-Lymphocytes / Q000276:immunology; D002869:Chromosome Aberrations; D002872:Chromosome Deletion; D025063:Chromosome Disorders; D005260:Female; D005803:Genes, Immunoglobulin; D006801:Humans; D007072:Immunoglobulin D / Q000235:genetics*; D007145:Immunoglobulin kappa-Chains / Q000235:genetics; D007146:Immunoglobulin lambda-Chains / Q000235:genetics*; D010954:Plasmacytoma / Q000235:genetics / Q000276:immunology*
chemical_list: D007072:Immunoglobulin D; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains
vernacular_title: Ungewöhnliche Immunglobulin-Gen-Rearrangements bei einem Patienten mit IgD/lambda-Plasmozytom.