Morley Ana M S, Verity David H, Meligonis George, Rose Geoffrey E
Moorfields Eye Hospital, London EC1V2PD, UK.
Orbit. 2009;28(6):425-9. doi: 10.3109/01676830903177427.
To describe two cases of orbital plasmablastic lymphoma (PBL), a recently defined aggressive large-cell lymphoma with a plasmacytic immunophenotype, typically occurring in the oral cavity of HIV+ patients. To compare their presentation, management, immunohistochemistry and prognosis with orbital diffuse large B-cell lymphoma (DLBCL).
Consecutive, comparative case series of patients presenting to a specialist orbital service with histologically proven PBL or DLBCL.
Two cases of PBL and 14 cases of DLBCL were identified. The former were 40-49-year-old men, one HIV+, presenting with marked orbital/sinonasal tumour without oral involvement. Both tumours were negative for B-cell markers (CD20, CD79a), showed a plasmacytic immunophenotype (positive for CD 138, Vs38c), and displayed a Ki67 index of 100%. Despite chemotherapy, both patients died within 1 year. Of the 14 patients with DLBCL, 57% were female, mean age 65 yrs, and none were immunocompromised. Only 7 (50%) had sinus involvement and 3 (21%) had prior systemic lymphoma. All were positive for B-cell markers, with a Ki67 index of 55%-80%. None died within 1 year of diagnosis.
PBL can present in the orbit and is more aggressive than DLBCL. It must be considered in the differential of orbital large-cell lymphoid tumours showing plasmablastic morphology or immunophenotype.
描述两例眼眶浆母细胞淋巴瘤（PBL），这是一种最近定义的具有浆细胞免疫表型的侵袭性大细胞淋巴瘤，通常发生于HIV阳性患者的口腔。将其临床表现、治疗、免疫组化及预后与眼眶弥漫性大B细胞淋巴瘤（DLBCL）进行比较。
对就诊于专科眼眶门诊且经组织学证实为PBL或DLBCL的患者进行连续、对比性病例系列研究。
共识别出2例PBL和14例DLBCL。前者为40 - 49岁男性，1例HIV阳性，表现为明显的眼眶/鼻窦肿瘤，无口腔受累。两种肿瘤的B细胞标志物（CD20、CD79a）均为阴性，呈浆细胞免疫表型（CD138、Vs38c阳性），Ki67指数为100%。尽管进行了化疗，两名患者均在1年内死亡。14例DLBCL患者中，57%为女性，平均年龄65岁，均无免疫功能低下。仅7例（50%）有鼻窦受累，3例（21%）曾有系统性淋巴瘤。所有患者B细胞标志物均为阳性，Ki67指数为55% - 80%。诊断后1年内无患者死亡。
PBL可发生于眼眶，且比DLBCL更具侵袭性。在鉴别表现为浆母细胞形态或免疫表型的眼眶大细胞淋巴肿瘤时必须考虑到该病。
