title: Correlation of immunophenotype with rearrangement of T cell antigen receptor beta and gamma genes in acute lymphoblastic leukemia of adults.
abstract: The structure of immunoglobulin heavy chain (IgH) and T cell antigen receptor (TCR) beta and gamma chain genes was studied in 38 cases of adult and two cases of childhood acute lymphoblastic leukemia (ALL). Seven cases of T-ALL all showed clonally rearranged TCR beta and gamma genes; only one of these also contained rearranged IgH genes. All precursor B cell ALLs and one case of unusual B cell ALL/lymphoma had clonally rearranged IgH genes, but a high proportion (22 of 32, 69%) of precursor B cell ALLs also had rearrangement of TCR beta and/or gamma genes. TCR beta gene rearrangement was less common in more mature precursor B cell ALL, expressing cytoplasmic IgM (pre-B-ALL) (0 of 5) than in other precursor B cell ALL cases (15 of 27). In the precursor B cell ALLs overall, 10 (32%) had rearrangement of both beta and gamma genes, while 7 (22%) had rearrangement of TCR gamma genes only. A further 5 (16%), all expressing one or more unusual immunophenotype markers, had TCR beta gene rearrangement without detectable gamma gene rearrangement. These observations, together with certain characteristics of constant-joining region usage of both TCR genes (a preference for rearrangement into the C beta 2 and C gamma 1 genes), distinguishes these "inappropriate" rearrangements from those found in T-ALL and suggests that they have arisen through a differentiation arrest which is not part of a normal T cell developmental program.
authors.affiliation: Department of Haematology, Royal Free Hospital and School of Medicine, London, U.K.
authors.forename: J D
authors.initials: JD
authors.lastname: Norton
authors.forename: D
authors.initials: D
authors.lastname: Campana
authors.forename: A V
authors.initials: AV
authors.lastname: Hoffbrand
authors.forename: G
authors.initials: G
authors.lastname: Janossy
authors.forename: E
authors.initials: E
authors.lastname: Couston-Smith
authors.forename: H
authors.initials: H
authors.lastname: Jani
authors.forename: J C
authors.initials: JC
authors.lastname: Yaxley
authors.forename: H G
authors.initials: HG
authors.lastname: Prentice
mesh_terms: D000208:Acute Disease; D000328:Adult; D001402:B-Lymphocytes; D005796:Genes; D006801:Humans; D007125:Immunogenetics; D007945:Leukemia, Lymphoid / Q000235:genetics* / Q000276:immunology; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000235:genetics*; D011995:Recombination, Genetic
chemical_list: D011948:Receptors, Antigen, T-Cell