Fornari Alessandro, Piva Roberto, Chiarle Roberto, Novero Domenico, Inghirami Giorgio
Department of Pathology and Center for Experimental Research and Medical Studies (CeRMS), University of Torino, Italy.
Hematol Oncol. 2009 Dec;27(4):161-70. doi: 10.1002/hon.897.
Anaplastic large cell lymphoma (ALCL) is a subtype of peripheral T-cell lymphoma (PTCL) first described in 1985 as a lymphoid malignancy characterized by marked cellular pleomorphism, propensity to grow cohesively, tendency to invade lymph node sinuses and diffuse expression of CD30 1. The discovery of the t(2;5), involving the anaplastic lymphoma kinase (ALK) gene on chromosome 2 and the nucleophosmin (NPM) gene on chromosome 5 in the majority of systemic ALCL, has soon pointed out that ALCL is a clinically and biologically heterogeneous disease. While ALK-positive (ALK+) ALCL is usually characterized by onset in children and young adults and better prognosis, epidemiology, poor outcome and possibly genetic defects of ALK-negative (ALK-) ALCL suggest that this neoplasms should be considered an independent pathological entity. The aim of this review is to illustrate clinical features, histology, immunophenotype, genetics and biology of ALCL and discuss possible relationship(s) among different T-non-Hodgkin lymphoma (T-NHL).
间变性大细胞淋巴瘤（ALCL）是外周T细胞淋巴瘤（PTCL）的一种亚型，于1985年首次被描述为一种淋巴恶性肿瘤，其特征为明显的细胞多形性、倾向于紧密生长、倾向于侵犯淋巴结窦以及CD30的弥漫性表达。在大多数系统性ALCL中发现了涉及2号染色体上的间变性淋巴瘤激酶（ALK）基因和5号染色体上的核磷蛋白（NPM）基因的t(2;5)，这很快表明ALCL是一种临床和生物学上异质性的疾病。虽然ALK阳性（ALK+）ALCL通常表现为儿童和年轻人发病且预后较好，但ALK阴性（ALK-）ALCL的流行病学、不良结局以及可能的基因缺陷表明，这种肿瘤应被视为一个独立的病理实体。本综述的目的是阐述ALCL的临床特征、组织学、免疫表型、遗传学和生物学，并讨论不同T细胞非霍奇金淋巴瘤（T-NHL）之间可能的关系。
