CD30-positive anaplastic large cell lymphoma in childhood: expression of p80npm/alk and absence of Epstein-Barr virus

Mod Pathol. 1997 Mar;10(3):210-5.


CD30(+)-anaplastic large cell lymphoma (ALCL) is a distinct clinicopathologic entity of non-Hodgkin's lymphoma that is immunologically heterogeneous. Bimodal age distribution, a nonrandom chromosome abnormality [t(2;5)(p23;q35)] that produces a chimeric protein p80npm/alk, and a variable (5-47%) association with Epstein-Barr virus (EBV) have been reported. We reviewed 36 cases of ALCL (19 were children < 20 yr and 17 were adults) by focusing on the presence or absence of p80npm/alk protein and EBV. Immunophenotyping studies were performed on frozen and/or paraffin sections before initiation of chemotherapy. The p80 protein was immunohistochemically examined, and EBV-encoded RNA transcripts were detected by in situ hybridization. Among 19 cases in children, 13 cases had a T-cell and 6 cases had a null-cell phenotype. p80 Was detected in 16 (84.2%) of 19 cases in children and 3 (17.6%) of 17 cases in adults. All of the 19 cases of p80-positive ALCL were positive for epithelial membrane antigen, regardless of age. EBV genome was not detected in any of 19 cases in children and in only 2 of 15 cases in adults. ALCL in childhood seems to constitute a homogeneous entity characterized by expression of p80 and absence of EBV. The association of EBV is also infrequent in adult ALCL among Japanese patients.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Female
  • Herpesvirus 4, Human / isolation & purification*
  • Humans
  • Immunohistochemistry
  • Immunophenotyping
  • In Situ Hybridization
  • Lymphoma, Large-Cell, Anaplastic / metabolism
  • Lymphoma, Large-Cell, Anaplastic / pathology*
  • Lymphoma, Large-Cell, Anaplastic / virology
  • Male
  • Middle Aged
  • Neoplasm Proteins / metabolism*


  • Neoplasm Proteins
  • protein p80