ALK-positive lymphoma:: A single disease with a broad spectrum of morphology
ALK-positive lymphoma:: A single disease with a broad spectrum of morphology
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DOI：
10.1182/blood.v91.6.2076
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发表时间：
1998-03-15
期刊：
影响因子：
20.3
通讯作者：
Delsol, G
中科院分区：
文献类型：
--
作者：
Benharroch, D;Meguerian-Bedoyan, Z;Delsol, G
与间变性大细胞淋巴瘤 (ALCL) 相关的 t(2;5)(p23;q35) 易位导致嵌合 NPM-ALK 蛋白的表达，该蛋白可通过 ALK1 单克隆抗体检测到。本报告描述了 123 例均表达 ALK 蛋白的淋巴瘤的形态学和表型谱。结果提供了强有力的证据，表明之前报告中描述的代表 ALCL 可能亚型的 ALCL 形态模式，例如普通型、淋巴组织细胞型或小细胞模式，是同一疾病实体的形态变异。所有这些形态学模式都可以在该系列中找到，并且在一些患者中，不同亚型在单次活检中共存，或者在单个患者的连续活检中发现。这些形态学亚型之间的联系因所有病例中都存在高度特征性的大细胞（具有偏心核和嗜酸性核旁区域）而进一步加强。我们认为该细胞可被视为 ALK 阳性淋巴瘤的主要区别特征。这些肿瘤的另一个特征是在大量病例中观察到肿瘤细胞浸润的血管周围模式。除 ALK 蛋白外，所有肿瘤均表达上皮膜抗原并缺乏 CD15，这些特征可能有助于区分 ALCL 与霍奇金病。在大多数病例 (84%) 中，恶性细胞显示出 ALK1 的细胞质和核染色，因此可能携带 2;5 易位，但在少数情况下染色仅限于细胞质，表明除 t(2;5) 以外的易位可能诱导 ALK 蛋白的表达。我们从这项研究中得出结论，ALK 阳性肿瘤代表了一个独特的实体。由于其形态通常既不是间变性也不是大细胞，我们建议今后将其称为 ALK 淋巴瘤。 (C) 1998 年，美国血液学会。
The t(2;5)(p23;q35) translocation, associated with anaplastic large-cell lymphoma (ALCL), results in the expression of a chimeric NPM-ALK protein that can be detected by the ALK1 monoclonal antibody. This report describes the morphologic and phenotypic spectrum of 123 cases of lymphoma that all express ALK protein. The results provide strong evidence that the morphologic patterns of ALCL described in previous reports as representing possible subtypes of ALCL, eg, common type, lymphohistiocytic, or small cell patterns, are morphologic variants of the same disease entity. All of these morphologic patterns could be found within this series, and in some patients different subtypes coexisted in a single biopsy or were found in successive biopsies from a single patient. The link between these morphologic subtypes is further reinforced by the presence in all cases of a highly characteristic large cell, with an eccentric nucleus and an eosinophilic paranuclear region. We suggest that this cell can be considered as a major distinguishing feature of ALK-positive lymphomas. Another characteristic of these tumors was the perivascular pattern of neoplastic cell infiltration seen in a significant number of cases. In addition to ALK protein, all tumors expressed epithelial membrane antigen and lacked CD15, features that may be of value in differentiating ALCL from Hodgkin's disease. In the majority of cases (84%), malignant cells showed both a cytoplasmic and nuclear staining for ALK1 and thus presumably carried the 2;5 translocation, but staining was restricted to the cytoplasm in a few cases, suggesting that translocations other than t(2;5) may induce expression of ALK protein. We conclude from this study that ALK-positive neoplasms represent a distinct entity. Because their morphology is often neither anaplastic nor large cell, we suggest that they should henceforward he referred to as ALK lymphomas. (C) 1998 by The American Society of Hematology.