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Anaplastic large cell lymphomas expressing the novel chimeric protein
p80NPM/ALK: a distinct clinicopathologic entity
M Shiota, S Nakamura, R Ichinohasama, M Abe, T Akagi, M Takeshita, N Mori, J Fujimoto, J Miyauchi and A Mikata
Department of Pathology, University of Tokyo, Japan.
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.
Volume 86,
Issue 5,
pp. 1954-1960,
09/01/1995
Copyright © 1995 by The American Society of Hematology
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