Cooper Medical School of Rowan University, 401 Broadway Ave, Camden, NJ, 08103, USA.
Division of Otolaryngology, Department of Surgery, Cooper University Hospital, 1 Cooper Plaza, Camden, NJ, 08103, USA.
Head Neck Pathol. 2020 Dec;14(4):1129-1133. doi: 10.1007/s12105-020-01135-1. Epub 2020 Jan 29.
Double-hit lymphoma (DHL) is a unique subtype of non-Hodgkin lymphoma characterized by atleast two rearrangements involving MYC, BLC2, and/or BCL6. These lymphomas are uncommon and aggressive, responding poorly to typical chemotherapy regimens. Lymphomas rarely arise from the oral cavity or tonsils, and those presenting as a neck mass are predominantly diffuse large B-cell lymphoma. To date, primary DHL of the tonsils has yet to be described in the literature. Here, we report a case of a 44 year-old male patient with well-controlled human immunodeficiency virus (HIV) who presented with a sore throat. He subsequently developed acute respiratory compromise due to a rapidly enlarging tonsillar mass. Pathologic and genetic analysis confirmed the presence of BCL6 and MYC rearrangements suggestive of DHL of the tonsils. In a young patient with HIV and a neck mass, it is essential that lymphoma remains on the list of differential diagnoses as prompt diagnosis and treatment may prevent complications from its rapid expansion.
双打击淋巴瘤(DHL)是一种独特的非霍奇金淋巴瘤亚型,其特征为至少涉及 MYC、BLC2 和/或 BCL6 的两种重排。这些淋巴瘤较为罕见且侵袭性强,对典型的化疗方案反应不佳。淋巴瘤很少起源于口腔或扁桃体,而表现为颈部肿块的则主要为弥漫性大 B 细胞淋巴瘤。迄今为止,扁桃体原发的 DHL 在文献中尚未被描述。在此,我们报告了一例 44 岁男性 HIV 感染者,因咽痛就诊。随后,由于扁桃体肿块迅速增大,他出现急性呼吸窘迫。病理和基因分析证实存在 BCL6 和 MYC 重排,提示为扁桃体 DHL。在 HIV 感染者和颈部肿块的年轻患者中,淋巴瘤必须作为鉴别诊断之一,因为及时诊断和治疗可能防止其快速扩展引起的并发症。