Chazerain P, Meyer O, Kaplan G, Brissaud P, Delmer A, Zufferey P, Kahn M F
Service de Rhumatologie, Hôpital Bichat, Paris.
Ann Med Interne (Paris). 1995;146(4):223-5.
The risk of malignant B cell lymphoma is increased in Sjögren's syndrome (SS). Orbital localization seems infrequent. We report 4 cases of malignant lymphoma (ML) occurring in 4 women aged 47 to 77 years, with primary SS in 3 cases, located to the conjunctiva in 2 cases, the lacrymal gland in 1 case and the eyelid in 1 case. The interval between the diagnosis of SS and orbital ML varied from 6 months to 15 years. All 4 lymphomas were of the B cell type, low histopathologic grade, with monoclonal gammopathy in 1 case. Extraocular lymphoma was initially present in 1 case. ML remained localized in 2 cases with a follow-up of 4 and 6 years. Two patients treated by excisional biopsy alone are in complete remission 3 and 6 years later. The 2 other patients treated with orbital radiotherapy and chemotherapy died rapidly (transformation into a high grade malignancy in 1 case). We conclude that clinical, immunopathologic features, as well as prognosis and treatment of ocular adnexa ML in SS are similar to those of primary ML without SS.
干燥综合征（SS）患者发生恶性B细胞淋巴瘤的风险增加。眼眶定位似乎并不常见。我们报告了4例发生在47至77岁女性中的恶性淋巴瘤（ML），其中3例为原发性SS，2例位于结膜，1例位于泪腺，1例位于眼睑。SS诊断与眼眶ML之间的间隔时间从6个月到15年不等。所有4例淋巴瘤均为B细胞型，组织病理学分级低，1例伴有单克隆丙种球蛋白病。1例最初表现为眼外淋巴瘤。2例ML在随访4年和6年后仍局限于局部。仅接受切除活检治疗的2例患者在3年和6年后完全缓解。另外2例接受眼眶放疗和化疗的患者很快死亡（1例转变为高级别恶性肿瘤）。我们得出结论，SS中眼附属器ML的临床、免疫病理特征以及预后和治疗与无SS的原发性ML相似。
