Anaplastic large cell lymphomas expressing the novel chimeric protein p80(NPM/ALK): A distinct clinicopathologic entity

M. Shiota, S. Nakamura, R. Ichinohasama, M. Abe, T. Akagi, M. Takeshita, N. Mori, J. Fujimoto, J. Miyauchi, A. Mikata, K. Nanba, T. Takami, H. Yamabe, Y. Takano, T. Izumo, T. Nagatani, N. Mohri, K. Nasu, H. Satoh

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329 Citations (Scopus)

Abstract

Anaplastic large cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma characterized by the CD30+ large neoplastic cells and sometimes carries a t(2;5)(p23;q35). Recently, we found a novel hyperphosphorylated 80- kD protein tyrosine kinase, p80, in ALCLs with t(2;5). Subsequent cDNA cloning showed 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, Science 263:1281, 1994). 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. Clinicopathologic comparison between p80-positive and -negative ALCLs showed that p80-positive cases occurred in a far younger patient age group (16.2 ± 12.9 years; p80- negative cases, 51.0 ± 22.3 years; P < .0001) and the patients showed a far better 5-year survival rate (79.8%; p80-negative group, 32.9%; P < .01). These data showed that p80-positive ALCL is a distinct entity both clinically and pathogenetically and should be differentiated from p80-negative ALCL.

Original languageEnglish
Pages (from-to)1954-1960
Number of pages7
JournalBlood
Volume86
Issue number5
DOIs
Publication statusPublished - 1995
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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