Shanghai Key Laboratory of Sleep Disordered Breathing, Department of Otolaryngology-Head and Neck Surgery, Otolaryngology Institute of Shanghai JiaoTong University, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Department of Neonatology, Children's Hospital of Soochow University, Suzhou, China.
Front Immunol. 2023 Jan 12;13:1062261. doi: 10.3389/fimmu.2022.1062261. eCollection 2022.
Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease with a predisposition towards autoimmunity and lymphoproliferative diseases. Non-Hodgkin lymphoma (NHL) is reported to be the predominant form of malignant tumor in WAS sufferers. Diffuse large B-cell lymphoma (DLBCL) is one of the most common types of NHL while it is uncommon to occur in paranasal sinuses and especially when associated with WAS. In this article, we report a unique case of WAS associated with DLBCL in paranasal sinuses and review the major publications of WAS-related lymphomas that occurred in the head and neck area. This study extends the available therapies for WAS-related lymphomas and emphasizes the significance of recognition for sinonasal lymphomas in WAS patients presenting with sinusitis.
威斯科特-奥尔德里奇综合征(WAS)是一种罕见的原发性免疫缺陷病,易发生自身免疫和淋巴增殖性疾病。据报道,非霍奇金淋巴瘤(NHL)是 WAS 患者中主要的恶性肿瘤形式。弥漫性大 B 细胞淋巴瘤(DLBCL)是 NHL 中最常见的类型之一,而在鼻窦中发生则较为罕见,特别是与 WAS 相关时更为罕见。本文报告了一例鼻窦弥漫性大 B 细胞淋巴瘤与 WAS 相关的独特病例,并回顾了头颈部发生的与 WAS 相关的淋巴瘤的主要文献。本研究拓展了 WAS 相关淋巴瘤的现有治疗方法,并强调了在出现鼻窦炎的 WAS 患者中识别鼻窦淋巴瘤的重要性。