Department of Pathology , University of Virginia Health, Charlottesville, VA, 22903, United States.
Department of Pathology , University of Virginia Health, Charlottesville, VA, 22903, United States.
Semin Diagn Pathol. 2023 Nov;40(6):392-400. doi: 10.1053/j.semdp.2023.06.002. Epub 2023 Jun 22.
Mature non-Hodgkin lymphomas (NHLs) of the pediatric and young adults(PYA), including Burkitt lymphoma (BL), diffuse large B cell lymphoma (DLBCL), high-grade B cell lymphoma (HGBCL), primary mediastinal large B cell lymphoma (PMBL) and anaplastic large cell lymphoma (ALCL), generally have excellent prognosis compared to the adult population. BL, DLBCL and HGBCL are usually of germinal center (GCB) origin in the PYA population. PMBL neither belongs to the GCB nor the activated B cell subtype and is associated with a poorer outcome than BL or DLBCL of comparable stage. Anaplastic large cell lymphoma is the most frequent peripheral T cell lymphoma occurring in the PYA and accounts for 10-15% of childhood NHL. Most pediatric ALCL, unlike in the adult, demonstrate expression of anaplastic lymphoma kinase (ALK). In recent years, the understanding of the biology and molecular features of these aggressive lymphomas has increased tremendously. This has led to reclassification of newer PYA entities including Burkitt-like lymphoma with 11q aberration. In this review, we will discuss the current progress discovered in frequently encountered aggressive NHLs in the PYA, highlighting the clinical, pathologic and molecular features that aid in the diagnosis of these aggressive lymphomas. We will be updating the new concepts and terminologies used in the new classification systems.
儿童和青年（PYA）成熟非霍奇金淋巴瘤（NHL），包括伯基特淋巴瘤（BL）、弥漫性大 B 细胞淋巴瘤（DLBCL）、高级别 B 细胞淋巴瘤（HGBCL）、原发性纵隔大 B 细胞淋巴瘤（PMBL）和间变性大细胞淋巴瘤（ALCL），与成人患者相比，一般预后较好。在 PYA 人群中，BL、DLBCL 和 HGBCL 通常起源于生发中心（GCB）。PMBL 既不属于 GCB 也不属于活化 B 细胞亚型，其预后比可比阶段的 BL 或 DLBCL 差。间变性大细胞淋巴瘤是 PYA 中最常见的外周 T 细胞淋巴瘤，占儿童 NHL 的 10-15%。与成人不同，大多数儿科间变性大细胞淋巴瘤（ALCL）表达间变性淋巴瘤激酶（ALK）。近年来，对这些侵袭性淋巴瘤的生物学和分子特征的认识有了极大的提高。这导致了对包括 11q 异常的伯基特样淋巴瘤在内的新型 PYA 实体的重新分类。在这篇综述中，我们将讨论在 PYA 中常见的侵袭性 NHL 中发现的最新进展，强调有助于诊断这些侵袭性淋巴瘤的临床、病理和分子特征。我们将更新新分类系统中使用的新概念和术语。
