Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets

Am J Clin Pathol. 2002 Feb;117(2):252-8. doi: 10.1309/08DJ-GPBH-H0VR-RC6F.

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.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Antigens, CD / biosynthesis
  • Antigens, Differentiation, Myelomonocytic / biosynthesis
  • Bone Marrow / pathology
  • CD13 Antigens / biosynthesis
  • Chromosome Aberrations
  • Chromosomes, Human, Pair 2
  • Cytogenetic Analysis
  • Female
  • Follow-Up Studies
  • Gene Rearrangement, T-Lymphocyte / genetics
  • Humans
  • Immunophenotyping
  • Male
  • Middle Aged
  • Polymerase Chain Reaction
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / immunology*
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / metabolism
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / pathology*
  • Receptors, Antigen, T-Cell / biosynthesis
  • Receptors, Antigen, T-Cell / genetics
  • Sialic Acid Binding Ig-like Lectin 3
  • Stem Cells / immunology*
  • Stem Cells / metabolism
  • Stem Cells / pathology*
  • Survival Rate

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD33 protein, human
  • Receptors, Antigen, T-Cell
  • Sialic Acid Binding Ig-like Lectin 3
  • CD13 Antigens