title: Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets.
abstract: We analyzed the clinicopathologic and molecular findings in 26 adults (age 16-72 years) with T-cell acute lymphoblastic leukemia (T-ALL) and observed features that correlated with age. Patients older than 60 years (n = 5) had a low frequency of hepatosplenomegaly (0 [0%]), anterior mediastinal mass (1 [20%]), and lymphadenopathy (2 [40%]), and completely responded to chemotherapy (4 of 4). The T-ALL in this group commonly expressed myeloid antigens (4 [80%]), had lineage-inappropriate gene rearrangements (2/3 [67%]) and chromosome 2 deletion (3/4 [75%]), and exclusively used the V(III) or V(IV) families of the T-cell receptor (TCR) gamma gene. In comparison, patients 16 to 60 years old (n = 21) more commonly had an anterior mediastinal mass (8 [38%]), hepatosplenomegaly (10 [48%]), and lymphadenopathy (16 [76%]). The tumors in these patients commonly used the TCR gamma gene VI or V(II) families (17/25 total rearrangements [68%]). Myeloid antigen expression (5 [24%]) and lineage inappropriate gene rearrangements (4/15 [27%]) were uncommon. Within this group, CD1a expression correlated with age 28 to 60 years. These results illustrate considerable age-related heterogeneity in adult T-ALL, which may reflect differences in tumor cell maturation.
authors.affiliation: Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
authors.forename: Mihaela
authors.initials: M
authors.lastname: Onciu
authors.forename: Raymond
authors.initials: R
authors.lastname: Lai
authors.forename: Francisco
authors.initials: F
authors.lastname: Vega
authors.forename: Carlos
authors.initials: C
authors.lastname: Bueso-Ramos
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D015703:Antigens, CD / Q000096:biosynthesis; D015214:Antigens, Differentiation, Myelomonocytic / Q000096:biosynthesis; D001853:Bone Marrow / Q000473:pathology; D018826:CD13 Antigens / Q000096:biosynthesis; D002869:Chromosome Aberrations; D002889:Chromosomes, Human, Pair 2; D020732:Cytogenetic Analysis; D005260:Female; D005500:Follow-Up Studies; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D011948:Receptors, Antigen, T-Cell / Q000096:biosynthesis / Q000235:genetics; D063268:Sialic Acid Binding Ig-like Lectin 3; D013234:Stem Cells / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D015996:Survival Rate
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; C571163:CD33 protein, human; D011948:Receptors, Antigen, T-Cell; D063268:Sialic Acid Binding Ig-like Lectin 3; D018826:CD13 Antigens