Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity.
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.
Shiota
M
M
Department of Pathology, University of Tokyo, Japan.
Nakamura
S
S
Ichinohasama
R
R
Abe
M
M
Akagi
T
T
Takeshita
M
M
Mori
N
N
Fujimoto
J
J
Miyauchi
J
J
Mikata
A
A
Nanba
K
K
Takami
T
T
Yamabe
H
H
Takano
Y
Y
Izumo
T
T
Nagatani
T
T
Mohri
N
N
Nasu
K
K
Satoh
H
H
Katano
H
H
Fujimoto
J
J
Yamamoto
T
T
Mori
S
S
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
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