Clinic of Hematology, Clinical Center of Serbia, Dr. Koste Todorovica 2, 11000 Belgrade, Republic Serbia.
Med Oncol. 2012 Sep;29(3):2207-11. doi: 10.1007/s12032-011-0022-x. Epub 2011 Jul 14.
Clinical features of 40 lymphoproliferative neoplasm patients in the setting of systemic autoimmune diseases managed in the Clinic of Hematology during 1994-2006 were analyzed retrospectively. The classification of systemic autoimmune disease patients was as follows: 15 systemic lupus erythematosus--SLE, 11 rheumatoid arthritis--RA, 12 Sjögren's syndrome--SS, 1 scleroderma, and 1 dermatomyositis. Patients comprised 31 women and 9 men of mean age 55 years (range 33-76). Systemic autoimmune diseases preceeded the development of lymphoproliferative neoplasms in 37/40 (92.5%) patients. Mean latency period between the onset of systemic autoimmune diseases and lymphoproliferative neoplasms occurrence was significantly longer in RA (113 months) than in SLE (75 months) and SS patients (65 months)--P < 0.05. The most frequent lymphoproliferative neoplasms were non-Hodgkin's lymphoma--NHL (35/40; 88%), diffuse large B-cell lymphoma (DBCL)--12 (34%), follicular lymphoma (FC)--7 (20%), small lymphocytic (SL), and marginal zone lymphoma (MZL)--5 (14%) each. The primary site of NHL was extranodal in 18/35 (51.5%) cases. Advanced disease on diagnosis (III + IV clinical stages), constitutional symptoms, and bulky disease were diagnosed in 27/35 (77%), 26/35 (74%), and 3/35 (8.5%) patients, respectively. The overall survival (OS) was as follows (months): DBCL-12, FC-63, SLL-60, and MZL-48. There was no association between the lymphoproliferative neoplasm histological subtype and the systemic autoimmune diseases type or antirheumatic treatment P > 0.05. Our findings are in line with earlier reports showing a high proportion of patients with advanced disease, constitutional symptoms, extranodal manifestations, high grade histology, and low OS in the systemic autoimmune diseases setting.
回顾性分析了 1994 年至 2006 年期间在血液科诊所接受治疗的 40 例系统性自身免疫性疾病合并淋巴增生性肿瘤患者的临床特征。系统性自身免疫性疾病患者的分类如下：15 例系统性红斑狼疮（SLE）、11 例类风湿关节炎（RA）、12 例干燥综合征（SS）、1 例硬皮病和 1 例皮肌炎。患者包括 31 名女性和 9 名男性，平均年龄为 55 岁（范围 33-76 岁）。在 40 例患者中，37 例（92.5%）系统性自身免疫性疾病先于淋巴增生性肿瘤发生。RA（113 个月）与 SLE（75 个月）和 SS 患者（65 个月）相比，系统性自身免疫性疾病和淋巴增生性肿瘤发生之间的潜伏期明显更长，P<0.05。最常见的淋巴增生性肿瘤是非霍奇金淋巴瘤（NHL）（35/40；88%）、弥漫性大 B 细胞淋巴瘤（DBCL）（12/35；34%）、滤泡性淋巴瘤（FC）（7/35；20%）、小淋巴细胞性（SLL）和边缘区淋巴瘤（MZL）（5/35；14%）。35 例 NHL 中，18 例（51.5%）为结外原发。27/35（77%）、26/35（74%）和 3/35（8.5%）患者分别诊断为晚期疾病、全身症状和肿块疾病。总体生存率（OS）如下：DBCL-12、FC-63、SLL-60 和 MZL-48。淋巴增生性肿瘤的组织学亚型与系统性自身免疫性疾病类型或抗风湿治疗之间无相关性，P>0.05。我们的研究结果与早期报告一致，即显示在系统性自身免疫性疾病背景下，患者晚期疾病、全身症状、结外表现、高级别组织学和低 OS 的比例较高。
