Arai Honoka, Iso Hisako, Arai Yukihiro, Tadokoro Jiro, Nakamura Yuko, Yamagata Tetsuya, Mitani Kinuko
Department of Hematology, Dokkyo Medical University School of Medicine, Japan.
Rinsho Ketsueki. 2009 Feb;50(2):97-101.
Malignant lymphoma (ML) is frequently associated with several forms of collagen diseases such as Sjören syndrome, systemic lupus erythematodes, and rheumatoid arthritis. However, the occurrence of ML in systemic sclerosis (SSc) patients has rarely been reported. Here we report an SSc patient who developed mediastinal (thymic) large B-cell lymphoma (MLBCL). A 31-year-old woman was diagnosed as having SSc in August 2007. The patient was treated with low-dose prednisolone (10 mg/day) without any effect. One year after the diagnosis, chest computed tomography-scan demonstrated thymic tumor in the anterior mediastinum. Thymectomy was performed, and a pathohistological diagnosis of MLBCL was established. Immunohistochemical analysis demonstrated that the tumor cells were positive for CD45 and CD20, but negative for CD30 and EBV-encoded RNA. The patient was treated with 6 courses of CHOP regimen, resulting in complete remission of lymphoma. This report describes the first SSc patient associated with MLBCL. SSc patients occasionally develop ML after a relatively short interval. Our case suggests that intensive monitoring for the development of ML is needed in newly diagnosed SSc patients.
恶性淋巴瘤（ML）常与多种胶原病相关，如干燥综合征、系统性红斑狼疮和类风湿关节炎。然而，系统性硬化症（SSc）患者发生ML的情况鲜有报道。在此，我们报告1例发生纵隔（胸腺）大B细胞淋巴瘤（MLBCL）的SSc患者。1名31岁女性于2007年8月被诊断为SSc。患者接受低剂量泼尼松龙（10毫克/天）治疗但无效。诊断1年后，胸部计算机断层扫描显示前纵隔有胸腺肿瘤。进行了胸腺切除术，并确立了MLBCL的病理组织学诊断。免疫组织化学分析显示肿瘤细胞CD45和CD20呈阳性，但CD30和EB病毒编码RNA呈阴性。该患者接受了6个疗程的CHOP方案治疗，淋巴瘤完全缓解。本报告描述了首例与MLBCL相关的SSc患者。SSc患者偶尔在相对较短的间隔后发生ML。我们的病例提示，新诊断的SSc患者需要密切监测ML的发生。
