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Blood, 1 November 2000, Vol. 96, No. 9, pp. 2993-3000
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Prognostic significance of CD56 expression for
ALK-positive and ALK-negative anaplastic
large-cell lymphoma of T/null cell phenotype
Ritsuro Suzuki,
Yoshitoyo Kagami,
Kengo Takeuchi,
Masahiro Kami,
Masataka Okamoto,
Ryo Ichinohasama,
Naoyoshi Mori,
Masaru Kojima,
Tadashi Yoshino,
Hirohiko Yamabe,
Mami Shiota,
Shigeo Mori,
Michinori Ogura,
Nobuyuki Hamajima,
Masao Seto,
Taizan Suchi,
Yasuo Morishima, and
Shigeo Nakamura
From the Division of Molecular Medicine, Department of
Hematology and Chemotherapy, Division of Epidemiology and Prevention,
and Department of Pathology and Genetics, Aichi Cancer Center, Nagoya;
the Department of Pathology, Faculty of Medicine, and Department of
Pathology, Institute of Medical Science, University of Tokyo, Tokyo;
Department of Hematology, Toranomon Hospital, Tokyo; Department of
Internal Medicine, Fujita Health University School of Medicine,
Toyoake; Department of Oral Pathology, Tohoku University School of
Medicine, Sendai; First Department of Pathology, Nagoya
University School of Medicine, Nagoya; Department of Pathology, Dokkyo
University School of Medicine, Tochigi; Department of Pathology,
Okayama University Medical School, Okayama; Laboratory of Anatomic
Pathology, Kyoto University School of Medicine, Kyoto,
Japan.
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.
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