Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity

Leukemia. 1997 Apr:11 Suppl 3:538-40.

Abstract

Some anaplastic large cell lymphomas (ALCLs) carry a specific chromosomal translocation, t(2;5)(p23;q35). Recently, we found a novel hyperphosphorylated 80-kDa protein tyrosine kinase, p80, in ALCLs with t(2;5). Subsequent cDNA cloning revealed p80 to be a fusion protein of two genes, the novel tyrosine kinase gene and the nucleophosmin gene, in accordance with the sequence of the NPM/ALK gene (Morris et al.). Meanwhile, the clinicopathologic features of p80-carrying ALCLs have remained unclear. Paraffin sections of 105 cases of ALCL were immunostained using anti-p80 antibody, and 30 of them were shown to express p80. Clinicopathological comparison between p80-positive and -negative ALCLs revealed that p80-positive cases occurred in a far younger patient age group and the patients showed a far better 5-year survival rate. These data showed that p80-positive ALCL is a distinct entity both clinically and pathogenetically, and should be differentiated from p80-negative ALCL.

MeSH terms

  • Adolescent
  • Adult
  • Anaplastic Lymphoma Kinase
  • Antigens, CD / analysis
  • Chromosome Mapping
  • Chromosomes, Human, Pair 2
  • Chromosomes, Human, Pair 5
  • Humans
  • Immunophenotyping
  • Lymphoma, Large B-Cell, Diffuse / genetics*
  • Lymphoma, Large B-Cell, Diffuse / immunology
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Middle Aged
  • Protein-Tyrosine Kinases / analysis
  • Protein-Tyrosine Kinases / biosynthesis*
  • Receptor Protein-Tyrosine Kinases
  • Recombinant Fusion Proteins / biosynthesis*
  • Survival Rate
  • Translocation, Genetic

Substances

  • Antigens, CD
  • Recombinant Fusion Proteins
  • p80(NPM-ALK) protein
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Protein-Tyrosine Kinases
  • Receptor Protein-Tyrosine Kinases