title: Immunoglobulin and T cell receptor gene configuration in acute lymphoblastic leukemia of infancy.
abstract: We examined immunoglobulin (Ig) heavy chain, K light chain, and T cell receptor (TCR) gamma and beta gene configuration in the leukemic cells from a series of infants aged less than 1 year with acute lymphoblastic leukemia (ALL). Each of these 11 cases demonstrated leukemic cell surface antigens that have been correlated with a B cell precursor phenotype. Of the 11, lymphoblasts of 4 retained the germline configuration of both Ig and TCR loci, whereas 7 had rearranged the Ig heavy chain gene. Two of these seven showed light chain gene rearrangement. TCB beta chain rearrangement had occurred in only one of the 11 patients' tumors. No TCR gamma chain rearrangements were identified. These results are in contrast to earlier studies of B cell precursor ALL in children in which Ig heavy chain gene rearrangements were evident in every case and approximately 40% showed Ig light chain rearrangement as well. In addition, 45% of cases of B cell precursor ALL of children had rearranged their gamma TCR genes, and 20% had rearranged beta. These data suggest that ALL in infancy represents an earlier stage of B cell development than is found in B cell precursor ALL of children. ALL in the infant age group has been associated with the worst prognosis of all patients with ALL. This study suggests that the disease in infants differs not only clinically, but also at the molecular genetic level, from the disease in children.
mesh_terms: D000208:Acute Disease; D002648:Child; D002675:Child, Preschool; D005796:Genes; D005838:Genotype; D006801:Humans; D007136:Immunoglobulins / Q000235:genetics*; D007223:Infant; D007231:Infant, Newborn; D007945:Leukemia, Lymphoid / Q000235:genetics* / Q000276:immunology / Q000503:physiopathology; D011971:Receptors, Immunologic / Q000235:genetics*; D013223:Statistics as Topic; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D007136:Immunoglobulins; D011971:Receptors, Immunologic