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

R. Suzuki, Y. Kagami, K. Takeuchi, M. Kami, M. Okamoto, R. Ichinohasama, N. Mori, M. Kojima, T. Yoshino, H. Yamabe, M. Shiota, S. Mori, M. Ogura, N. Hamajima, M. Seto, T. Suchi, Y. Morishima, S. Nakamura

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156 Citations (Scopus)


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. (C) 2000 by The American Society of Hematology.

Original languageEnglish
Pages (from-to)2993-3000
Number of pages8
Issue number9
Publication statusPublished - Nov 1 2000
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology


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