title: Prognostic significance of CD56 expression for ALK-positive and ALK-negative anaplastic large-cell lymphoma of T/null cell phenotype.
abstract: Anaplastic large cell lymphoma (ALCL) is a distinct entity of non-Hodgkin lymphoma, characterized by a proliferation of pleomorphic large lymphoid cells that express CD30. Recent studies have found that a subset of ALCL aberrantly expresses a chimeric anaplastic lymphoma kinase (ALK) protein as a result of t(2;5)(p23;q35) or variant translocations. ALK-positive ALCLs feature good prognosis, but some of them lead to poor outcomes. Since CD56 is expressed in some ALCLs, its clinical significance was examined in a series of T/null cell type ALCLs. Of 143 patients, 83 (58%) showed ALK-positive staining, and of 140 patients, 25 (18%) expressed CD56. The ALK-positive subgroup was characterized by a younger age of onset (P <.0001), lower serum lactate dehydrogenase level (P =.01), better performance status (P =.03), less frequent extranodal involvement (P =.01), lower international prognostic index (IPI) categories (P =.002), and superior survival (P =.0009) in comparison with the ALK-negative group, suggesting that ALK is a specific marker defining a distinct subtype. CD56(+) cases showed a significantly poor prognosis overall (P =.002) as well as in both ALK-positive and ALK-negative subgroups (P =.02 and P =.04, respectively). Multivariate analysis confirmed that CD56 is independent of other prognostic factors, including IPI. Although CD56(+) cases showed a higher incidence of bone involvement, no other differences in clinicopathologic parameters were found between the CD56(+) and CD56(-) groups. These findings suggest that CD56 is not a marker to identify a distinct subtype of ALCL, but a strong clinical prognostic factor. Effective therapeutic approaches should be explored for high-risk ALCL patients, who can be identified by means of a prognostic model, including CD56.
authors.affiliation: Division of Molecular Medicine, Department of Hematology and Chemotherapy, Nagoya, Japan. rsuzuki@aichicc.pref.aichi.jp
authors.forename: R
authors.initials: R
authors.lastname: Suzuki
authors.forename: Y
authors.initials: Y
authors.lastname: Kagami
authors.forename: K
authors.initials: K
authors.lastname: Takeuchi
authors.forename: M
authors.initials: M
authors.lastname: Kami
authors.forename: M
authors.initials: M
authors.lastname: Okamoto
authors.forename: R
authors.initials: R
authors.lastname: Ichinohasama
authors.forename: N
authors.initials: N
authors.lastname: Mori
authors.forename: M
authors.initials: M
authors.lastname: Kojima
authors.forename: T
authors.initials: T
authors.lastname: Yoshino
authors.forename: H
authors.initials: H
authors.lastname: Yamabe
authors.forename: M
authors.initials: M
authors.lastname: Shiota
authors.forename: S
authors.initials: S
authors.lastname: Mori
authors.forename: M
authors.initials: M
authors.lastname: Ogura
authors.forename: N
authors.initials: N
authors.lastname: Hamajima
authors.forename: M
authors.initials: M
authors.lastname: Seto
authors.forename: T
authors.initials: T
authors.lastname: Suchi
authors.forename: Y
authors.initials: Y
authors.lastname: Morishima
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000077548:Anaplastic Lymphoma Kinase; D015703:Antigens, CD / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D016403:Lymphoma, Large B-Cell, Diffuse / Q000145:classification / Q000276:immunology* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000032:analysis*; D020794:Receptor Protein-Tyrosine Kinases; D015996:Survival Rate; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D015703:Antigens, CD; D014408:Biomarkers, Tumor; D019002:CD56 Antigen; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases