Configuration of the immunoglobulin and T cell receptor gene regions in hairy cell leukemia and B-chronic lymphocytic leukemia.
The configuration of the immunoglobulin heavy and light chain genes and of the T cell receptor (TCR) beta region was examined in a series of patients with hairy cell leukemia (HCL) and compared with the findings in B cell chronic lymphocytic leukemia (B-CLL). In all cases of HCL and B-CLL studied a rearrangement of the immunoglobulin heavy chain loci coupled to a reorganization of the light chain genes was documented. However, in all HCL and all but one B-CLL the TCR beta gene region was in a germ-line configuration. These findings confirm that HCL is characterized by the expansion of relatively mature B cell elements with a molecular configuration similar to that of B-CLL and indicate that the reported expression of T cell related markers, particularly in B-CLL, is not coupled to a rearrangement of the TCR beta-chain gene. Analyses of the immunoglobulin gene regions in HCL represent an important diagnostic tool as well as a possible aid toward monitoring the response to treatment.
D001402:B-Lymphocytes / Q000502:physiology*; D004273:DNA, Neoplasm / Q000235:genetics; D005803:Genes, Immunoglobulin; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007145:Immunoglobulin kappa-Chains / Q000235:genetics; D007146:Immunoglobulin lambda-Chains / Q000235:genetics; D007943:Leukemia, Hairy Cell / Q000235:genetics*; D007945:Leukemia, Lymphoid / Q000235:genetics*; D011948:Receptors, Antigen, T-Cell / Q000235:genetics*
D004273:DNA, Neoplasm; D007143:Immunoglobulin Heavy Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D011948:Receptors, Antigen, T-Cell