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Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity
M Shiota1, S Nakamura, R Ichinohasama
1Department of Pathology, University of Tokyo, Japan.
Blood
|September 1, 1995
Summary
p80-positive anaplastic large cell lymphoma (ALCL) is a distinct subtype. These ALCL cases occur in younger patients and show significantly better survival rates compared to p80-negative ALCL.
Area of Science:
- Oncology
- Hematology
- Molecular Biology
Background:
- Anaplastic large cell lymphoma (ALCL) is a CD30+ non-Hodgkin's lymphoma, sometimes associated with the t(2;5) translocation.
- A novel hyperphosphorylated protein tyrosine kinase, p80, has been identified in ALCLs with t(2;5).
- p80 is a fusion protein of a novel tyrosine kinase gene and the nucleophosmin gene, consistent with the NPM/ALK gene.
Purpose of the Study:
- To investigate the clinicopathologic features of p80-carrying ALCL.
- To determine if p80 expression defines a distinct ALCL subtype.
Main Methods:
- Immunohistochemical staining using anti-p80 antibody on 105 ALCL paraffin sections.
- Clinicopathologic comparison between p80-positive and p80-negative ALCL cases.
Main Results:
- p80 expression was detected in 30 out of 105 ALCL cases.
- p80-positive ALCL cases were significantly younger (16.2 years) than p80-negative cases (51.0 years).
- p80-positive ALCL showed a better 5-year survival rate (79.8%) compared to p80-negative ALCL (32.9%).
Conclusions:
- p80-positive ALCL represents a distinct clinicopathologic and pathogenetic entity.
- Differentiating p80-positive ALCL from p80-negative ALCL is clinically important.
