Department of Pathology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Orbit. 2022 Oct;41(5):624-628. doi: 10.1080/01676830.2021.1904426. Epub 2021 Mar 29.
High-grade B-cell lymphoma (HGBL) with and and/or rearrangements, also known as a double-hit and triple-hit lymphoma, is an aggressive non-Hodgkin lymphoma affecting older adults. After formal recognition of this entity in the 2017 revision of the World Health Organization Classification of lymphoid neoplasms, only two well-documented cases of triple-hit lymphoma of the orbit appear in the literature. Herein, we describe a 70-year-old man with progressive diplopia, ophthalmoplegia, and rapidly enlarging temporal mass. Biopsy revealed a tumor morphologically consistent with HGBL, coexpressing CD20, CD10, BCL6, BCL2, and c-MYC on immunohistochemical analysis. Fluorescence in-situ hybridization showed rearrangements in and genes, confirming double-hit HGBL. Systemic workup revealed Ann Arbor stage IV disease. This report reviews the existing literature on ocular adnexal double-hit and triple-hit lymphoma and provides an update on the diagnostic ancillary studies, prognostic implications, and latest management for this aggressive hematolymphoid malignancy.
伴有 和/或 重排的高级别 B 细胞淋巴瘤（HGBL），也称为双打击和三打击淋巴瘤，是一种侵袭性非霍奇金淋巴瘤，影响老年人。在 2017 年世界卫生组织淋巴肿瘤分类修订版正式确认该实体后，文献中仅报道了两例明确记录的眼眶三打击淋巴瘤病例。在此，我们描述了一名 70 岁男性，表现为进行性复视、眼肌麻痹和颞部迅速增大的肿块。活检显示肿瘤形态符合 HGBL，免疫组织化学分析显示 CD20、CD10、BCL6、BCL2 和 c-MYC 共表达。荧光原位杂交显示 和 基因重排，证实为双打击 HGBL。系统检查显示安阿伯分期为 IV 期疾病。本报告回顾了眼部附属器双打击和三打击淋巴瘤的现有文献，并提供了对这种侵袭性血液淋巴恶性肿瘤的辅助诊断、预后意义和最新治疗的最新信息。
