Prognostic significance of CD56 expression for ALK-positive and ALK-negative anaplastic large-cell lymphoma of T/null cell phenotype

Citation
R. Suzuki et al., Prognostic significance of CD56 expression for ALK-positive and ALK-negative anaplastic large-cell lymphoma of T/null cell phenotype, BLOOD, 96(9), 2000, pp. 2993-3000
Citations number
81
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
BLOOD
ISSN journal
0006-4971 → ACNP
Volume
96
Issue
9
Year of publication
2000
Pages
2993 - 3000
Database
ISI
SICI code
0006-4971(20001101)96:9<2993:PSOCEF>2.0.ZU;2-B
Abstract
Anaplastic large cell lymphoma (ALCL) is a distinct entity of non-Hodgkin l ymphoma, characterized by a proliferation of pleomorphic large lymphoid cel ls that express CD30, Recent studies have found that a subset of ALCL aberr antly expresses a chimeric anaplastic lymphoma kinase (ALK) protein as a re sult of t(2; 5)(p23;q35) or variant translocations. ALK-positive ALCLs feat ure good prognosis, but some of them lead to poor outcomes. Since CD56 is e xpressed 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 st aining, and of 140 patients, 25 (18%) expressed CD56. The ALK-positive subg roup was characterized by a younger age of onset (P < .0001), lower serum l actate dehydrogenase level (P = .01), better performance status (P = .03), less frequent extranodal involvement (P = .01), lower international prognos tic 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 po or prognosis overall (P = .002) as well as in both ALK-positive and ALK-neg ative subgroups (P = .02 and P = .04, respectively). Multivariate analysis con-firmed 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 mark er 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, includ ing CD56. (C) 2000 by The American Society of Hematology.