Suzuki R, Kagami Y, Takeuchi K, Kami M, Okamoto M, Ichinohasama R, Mori N, Kojima M, Yoshino T, Yamabe H, Shiota M, Mori S, Ogura M, Hamajima N, Seto M, Suchi T, Morishima Y, Nakamura S
Division of Molecular Medicine, Department of Hematology and Chemotherapy, Nagoya, Japan.
Blood. 2000 Nov 1;96(9):2993-3000.
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.
间变性大细胞淋巴瘤（ALCL）是一种独特的非霍奇金淋巴瘤实体，其特征是表达CD30的多形性大淋巴细胞增殖。最近的研究发现，由于t(2;5)(p23;q35)或变异易位，一部分ALCL异常表达嵌合性间变性淋巴瘤激酶（ALK）蛋白。ALK阳性的ALCL预后良好，但其中一些会导致不良结局。由于CD56在一些ALCL中表达，因此在一系列T/null细胞型ALCL中研究了其临床意义。143例患者中，83例（58%）ALK染色呈阳性，140例患者中，25例（18%）表达CD56。与ALK阴性组相比，ALK阳性亚组的特征为发病年龄较轻（P<.0001）、血清乳酸脱氢酶水平较低（P=.01）、体能状态较好（P=.03）、结外受累较少（P=.01）、国际预后指数（IPI）类别较低（P=.002）以及生存率较高（P=.0009），这表明ALK是定义一个独特亚型的特异性标志物。CD56(+)病例总体预后明显较差（P=.002），在ALK阳性和ALK阴性亚组中均如此（分别为P=.02和P=.04）。多因素分析证实，CD56独立于其他预后因素，包括IPI。尽管CD56(+)病例骨受累发生率较高，但CD56(+)组和CD56(-)组在其他临床病理参数方面未发现差异。这些发现表明，CD56不是识别ALCL独特亚型的标志物，而是一个强有力的临床预后因素。对于可通过包括CD56在内的预后模型识别出的高危ALCL患者，应探索有效的治疗方法。
