Sabt Buthaina I, Al Yahyai Majda, Al-Mujaini Arwa A, Al-Mujaini Abdullah S
Department of Ophthalmology, Sultan Qaboos University Hospital, Muscat, Oman.
Department of Ophthalmology, Al-Nahda Hospital, Ministry of Health, Muscat, Oman.
Saudi J Ophthalmol. 2022 Feb 18;35(2):164-166. doi: 10.4103/1319-4534.337858. eCollection 2021 Apr-Jun.
Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4 level, and infiltration with IgG4-positive plasma cells. Ocular adnexal MZBLs are reported to arise in IgG4-related sclerosing dacryoadenitis, indicating a possible link between the two conditions. Here, we describe a 37-year-old Omani male who presented with right periorbital swelling and proptosis 4 years before presentation. He was diagnosed to have right orbital pseudotumor and exhibited good response to steroid therapy. However, 4 years later, rapid swelling of the right orbital mass was observed. The patient underwent lacrimal gland biopsy. Although the histology was consistent with IgG4-related disease, the infiltrating large atypical lymphoid cells showed that immunoglobulin light-chain restriction and dense lymphoplasmacytic infiltrate involving the soft tissue were seen. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells of the right lacrimal gland.
眼附属器边缘区B细胞淋巴瘤（MZBLs）占眼附属器淋巴瘤的大多数。免疫球蛋白G4（IgG4）相关疾病是最近提出的一种疾病实体，具有一些独特的临床病理特征，如受累器官肿大、血清IgG4水平升高以及IgG4阳性浆细胞浸润。据报道，眼附属器MZBLs发生于IgG4相关硬化性泪腺炎，提示这两种疾病之间可能存在联系。在此，我们描述一名37岁的阿曼男性，他在就诊前4年出现右侧眶周肿胀和眼球突出。他被诊断为右侧眼眶假瘤，对类固醇治疗反应良好。然而，4年后，观察到右侧眼眶肿物迅速肿胀。患者接受了泪腺活检。尽管组织学与IgG4相关疾病一致，但浸润的大的非典型淋巴细胞显示出免疫球蛋白轻链限制，并且在软组织中可见密集的淋巴浆细胞浸润。因此，他被诊断为右侧泪腺伴有大量IgG4阳性细胞的结外边缘区淋巴瘤。
