Mereu Elisabetta, Pellegrino Elisa, Scarfò Irene, Inghirami Giorgio, Piva Roberto
Department of Molecular Biotechnology and Health Sciences, Center for Experimental Research and Medical Studies, University of Torino, Torino, Italy.
Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA, USA.
Oncotarget. 2017 Mar 14;8(11):18525-18536. doi: 10.18632/oncotarget.14503.
Anaplastic Large Cell Lymphoma (ALCL) is a clinical and biological heterogeneous disease including systemic ALK positive and ALK negative entities. Whereas ALK positive ALCLs are molecularly characterized and readily diagnosed, specific immunophenotypic or genetic features to define ALK negative ALCL are missing, and their distinction from other T-cell non-Hodgkin lymphomas (T-NHLs) can be controversial. In recent years, great advances have been made in dissecting the heterogeneity of ALK negative ALCLs and in providing new diagnostic and treatment options for these patients. A new revision of the World Health Organization (WHO) classification promoted ALK negative ALCL to a definite entity that includes cytogenetic subsets with prognostic implications. However, a further understanding of the genetic landscape of ALK negative ALCL is required to dictate more effective therapeutic strategies specifically tailored for each subgroup of patients.
间变性大细胞淋巴瘤(ALCL)是一种临床和生物学上异质性的疾病,包括系统性ALK阳性和ALK阴性实体。虽然ALK阳性的ALCL在分子水平上有特征且易于诊断,但用于定义ALK阴性ALCL的特定免疫表型或基因特征尚缺失,并且将它们与其他T细胞非霍奇金淋巴瘤(T-NHL)区分开来可能存在争议。近年来,在剖析ALK阴性ALCL的异质性以及为这些患者提供新的诊断和治疗选择方面取得了巨大进展。世界卫生组织(WHO)分类的新修订将ALK阴性ALCL提升为一个明确的实体,其中包括具有预后意义的细胞遗传学亚组。然而,需要进一步了解ALK阴性ALCL的基因图谱,以制定专门针对每个患者亚组的更有效的治疗策略。