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

• Published in: Blood Vol. 96; no. 9; pp. 2993 - 3000
• Main Authors: Suzuki, Ritsuro, Kagami, Yoshitoyo, Takeuchi, Kengo, Kami, Masahiro, Okamoto, Masataka, Ichinohasama, Ryo, Mori, Naoyoshi, Kojima, Masaru, Yoshino, Tadashi, Yamabe, Hirohiko, Shiota, Mami, Mori, Shigeo, Ogura, Michinori, Hamajima, Nobuyuki, Seto, Masao, Suchi, Taizan, Morishima, Yasuo, Nakamura, Shigeo
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 01-11-2000; The Americain Society of Hematology
• Subjects: Adolescent; Adult; Age of Onset; Aged; Aged, 80 and over; Anaplastic Lymphoma Kinase; Antigens, CD - analysis; Biological and medical sciences; Biomarkers, Tumor - analysis; CD56 Antigen - analysis; Child; Child, Preschool; Female; Hematologic and hematopoietic diseases; Humans; Immunophenotyping; Infant; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - classification; Lymphoma, Large B-Cell, Diffuse - immunology; Lymphoma, Large B-Cell, Diffuse - mortality; Lymphoma, Large B-Cell, Diffuse - pathology; Male; Medical sciences; Middle Aged; Predictive Value of Tests; Prognosis; Protein-Tyrosine Kinases - analysis; Receptor Protein-Tyrosine Kinases; Survival Rate; T-Lymphocytes - immunology; Chromosomal aberration; Human; Prognosis; Immunophenotype; Enzyme; Transferases; Biological marker; Cytotoxicity; Malignant hemopathy; Large cell lymphoma; Non Hodgkin lymphoma; Abnormal chromosome B5; Kinase; Lymphoproliferative syndrome; Abnormal chromosome A2; T-Lymphocyte; Ki1 positive large cell anaplastic lymphoma; Abnormal chromosome; Null cell; Fusion protein; Chromosome translocation
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V96.9.2993

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 andP = .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.