title: Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity.
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.
authors.affiliation: Department of Pathology, University of Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Shiota
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
authors.forename: R
authors.initials: R
authors.lastname: Ichinohasama
authors.forename: M
authors.initials: M
authors.lastname: Abe
authors.forename: T
authors.initials: T
authors.lastname: Akagi
authors.forename: M
authors.initials: M
authors.lastname: Takeshita
authors.forename: N
authors.initials: N
authors.lastname: Mori
authors.forename: J
authors.initials: J
authors.lastname: Fujimoto
authors.forename: J
authors.initials: J
authors.lastname: Miyauchi
authors.forename: A
authors.initials: A
authors.lastname: Mikata
authors.forename: K
authors.initials: K
authors.lastname: Nanba
authors.forename: T
authors.initials: T
authors.lastname: Takami
authors.forename: H
authors.initials: H
authors.lastname: Yamabe
authors.forename: Y
authors.initials: Y
authors.lastname: Takano
authors.forename: T
authors.initials: T
authors.lastname: Izumo
authors.forename: T
authors.initials: T
authors.lastname: Nagatani
authors.forename: N
authors.initials: N
authors.lastname: Mohri
authors.forename: K
authors.initials: K
authors.lastname: Nasu
authors.forename: H
authors.initials: H
authors.lastname: Satoh
authors.forename: H
authors.initials: H
authors.lastname: Katano
authors.forename: J
authors.initials: J
authors.lastname: Fujimoto
authors.forename: T
authors.initials: T
authors.lastname: Yamamoto
authors.forename: S
authors.initials: S
authors.lastname: Mori
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D015703:Antigens, CD / Q000032:analysis / Q000096:biosynthesis; D002874:Chromosome Mapping; D002889:Chromosomes, Human, Pair 2; D002895:Chromosomes, Human, Pair 5; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D017730:Ki-1 Antigen / Q000032:analysis / Q000096:biosynthesis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000145:classification / Q000235:genetics* / Q000401:mortality / Q000473:pathology*; D008875:Middle Aged; D009687:Nuclear Proteins / Q000032:analysis / Q000096:biosynthesis*; D000090243:Nucleophosmin; D010750:Phosphoproteins / Q000032:analysis / Q000096:biosynthesis; D010766:Phosphorylation; D011505:Protein-Tyrosine Kinases / Q000032:analysis / Q000096:biosynthesis*; D011993:Recombinant Fusion Proteins / Q000032:analysis / Q000096:biosynthesis*; D015996:Survival Rate; D014178:Translocation, Genetic
chemical_list: D015703:Antigens, CD; D017730:Ki-1 Antigen; C000716967:NPM1 protein, human; D009687:Nuclear Proteins; D010750:Phosphoproteins; D011993:Recombinant Fusion Proteins; D000090243:Nucleophosmin; D011505:Protein-Tyrosine Kinases