Kinney M C, Kadin M E
Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Am J Clin Pathol. 1999 Jan;111(1 Suppl 1):S56-67.
Anaplastic large cell lymphoma (ALCL) is clinically and pathologically heterogeneous, leading to some difficulty in its diagnosis and acceptance as a specific entity. ALCL can be subdivided into different groups according to histologic features (pleomorphic, monomorphic, small cell predominant, Hodgkin's disease-related, and other less common variants), immunophenotype (T, null, B, and rarely B and T), and clinical features (systemic, primary cutaneous type, ALCL arising in HIV-positive patients, and ALCL occurring after another lymphoproliferative process, such as lymphomatoid papulosis, mycosis fungoides, and Hodgkin's disease. Cytogenetic studies and subsequent cloning of the translocation t(2;5) have shown a high degree of association with Ki-1-positive lymphoma but have revealed that this genetic abnormality is not specific for anaplastic morphologic features, is more common in the monomorphic and small cell variants, and is rare in primary cutaneous ALCL. It is likely that this t(2;5)-positive group is pathogenetically related. The purpose of this article is to review the pathologic and clinical spectrum of ALCL, including the borderline with Hodgkin's disease and lymphomatoid papulosis and to discuss the use of the t(2;5) in better defining a more specific molecular pathologic entity within this group of diseases with CD30 expression.
间变性大细胞淋巴瘤（ALCL）在临床和病理上具有异质性，这给其诊断以及作为一种特定实体被认可带来了一定困难。根据组织学特征（多形性、单形性、小细胞为主型、霍奇金病相关型以及其他较少见的变异型）、免疫表型（T细胞型、无免疫表型型、B细胞型，以及罕见的B细胞和T细胞混合型）和临床特征（系统性、原发性皮肤型、HIV阳性患者中发生的ALCL，以及在另一种淋巴增殖性疾病如淋巴瘤样丘疹病、蕈样肉芽肿和霍奇金病之后发生的ALCL），ALCL可被细分为不同组。细胞遗传学研究以及随后对t(2;5)易位的克隆显示，其与Ki-1阳性淋巴瘤高度相关，但也表明这种基因异常并非间变性形态特征所特有，在单形性和小细胞变异型中更常见，而在原发性皮肤ALCL中罕见。t(2;5)阳性组可能在发病机制上相关。本文的目的是综述ALCL的病理和临床谱，包括与霍奇金病和淋巴瘤样丘疹病的交界情况，并讨论t(2;5)在更好地界定这组具有CD30表达的疾病中更特异的分子病理实体方面的应用。
