Talal N, Aufdemorte T B, Kincaid W L, Sayers B S, Lynn J T
Clinical Immunology Section, Audie L. Murphy Memorial Veterans Hospital, San Antonio, TX.
J Autoimmun. 1988 Apr;1(2):171-84. doi: 10.1016/0896-8411(88)90024-8.
Two patients with Sjögren's syndrome (SS) who subsequently developed malignant B-cell lymphomas are reported in detail. The first patient had both benign- and malignant-appearing lymphoid infiltrates on the same submandibular gland specimen and was successfully treated with combined chemotherapy and irradiation. The second patient developed cutaneous lymphoid infiltrates difficult to diagnose by light microscopy but containing a monoclonal IgM-Kappa population revealed by immunoperoxidase staining and immunoglobulin gene rearrangement studies. Her lesions resolved rapidly and completely on cyclophosphamide, recurred rapidly when this drug was discontinued, and resolved again on a second course of cyclophosphamide which is currently maintained at 50 mg daily. Both patients are doing well without recurrence two and three years after initial treatment. This clinical experience is presented to emphasize: (1) the clinical use of molecular biologic techniques to define the earliest appearance of malignant transformation in Sjögren's syndrome, and (2) the successful outcome that can be achieved with prompt institution of appropriate treatment. The phenomenon of lymphoma development in SS is discussed with regard to immunoregulatory abnormalities predisposing to malignancy in the setting of autoimmune disease.
详细报告了两名随后发展为恶性B细胞淋巴瘤的干燥综合征（SS）患者。首例患者在同一颌下腺标本上既有良性又有恶性表现的淋巴浸润，经联合化疗和放疗成功治愈。第二例患者出现皮肤淋巴浸润，光镜下难以诊断，但免疫过氧化物酶染色和免疫球蛋白基因重排研究显示其中含有单克隆IgM-κ轻链群体。她的病变在环磷酰胺治疗后迅速完全消退，停药后迅速复发，再次给予环磷酰胺治疗（目前每日维持剂量为50mg）后又消退。两名患者在初始治疗后两年和三年均无复发，情况良好。介绍这一临床经验是为了强调：（1）分子生物学技术在临床上用于确定干燥综合征恶性转化最早表现的应用；（2）及时采用适当治疗可取得成功的结果。还讨论了干燥综合征中淋巴瘤发生的现象，涉及自身免疫性疾病背景下易发生恶性肿瘤的免疫调节异常。
