Ocular adnexal mucosa-associated lymphoid tissue lymphoma with polyclonal hypergammaglobulinemia
Ocular adnexal mucosa-associated lymphoid tissue lymphoma with polyclonal hypergammaglobulinemia
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DOI：
10.1016/j.ajo.2008.01.006
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发表时间：
2008-06-01
影响因子：
4.2
通讯作者：
Terasaki, Hiroko
中科院分区：
文献类型：
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作者：
Kubota, Toshinobu;Moritani, Suzuko;Terasaki, Hiroko
目得：确定原发性眼附属器粘膜相关淋巴组织（MALT）淋巴瘤伴多克隆高丙种球蛋白血症患者的特征。设计：病例系列研究。方法：在81例日本原发性眼附属器MALT淋巴瘤患者中，7例（9%）被诊断为多克隆高丙种球蛋白血症。患者临床数据包括自身免疫性疾病和传播史。从所有患者收集的外周血进行了分析，类风湿因子，可溶性白细胞介素2受体（sIL 2 R），免疫球蛋白的血清水平在诊断时和每次treatment.Results：7例多克隆高丙种球蛋白血症患者有类风湿因子，sIL-2 R，免疫球蛋白G（IgG），免疫球蛋白E（IgE）的血清水平升高在诊断时。1例患者有干燥综合征。6例患者（86%）在诊断时有MALT淋巴瘤或淋巴结病的扩散。淋巴结病患者的组织学检查不仅显示MALT淋巴瘤，而且还显示次级滤泡。尽管放疗后眼部病变完全消退，但7例患者的血清IgG、类风湿因子或sIL-2 R水平均无改善。在给予环磷酰胺/阿霉素/长春新碱，tine/泼尼松和/或利妥昔单抗的3名患者，所有三个显示出改善血清IgG，类风湿因子，和sIL-2的水平结论：眼附属器MALT淋巴瘤和多克隆高丙种球蛋白血症的患者有类风湿因子，sIL-2 R，和IgE的血清水平升高，和高扩散或淋巴结病。这些独特的特征可能与全身免疫失衡有关。
PURPOSE: To determine the characteristics of patients with primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma associated with polyclonal hypergammaglobulinemia.DESIGN: Case series study.METHODS: Among 81 Japanese patients with primary ocular adnexal MALT lymphoma, seven patients (9%) were diagnosed with polyclonal hypergammaglobulinemia. Patient clinical data included a history of autoimmune disease and dissemination. Peripheral blood collected from all patients was analyzed for serum levels of rheumatoid factor, soluble interleukin-2 receptor (sIL 2R), and immunoglobulins at the time of diagnosis and after each treatment.RESULTS: Seven patients with polyclonal hypergammaglobulinemia had elevated serum levels of rheumatoid factor, sIL-2R, immunoglobulin G (IgG), and immunoglobulin E (IgE) at the time of diagnosis. One patient had Sjogren syndrome. Six patients (86%) had a dissemination of the MALT lymphoma or lymphadenopathy at the time of diagnosis. Histopathologic examination of the patients with lymphadenopathy revealed not only MALT lymphoma but also secondary follicles. None of the seven patients showed improvement in serum levels of IgG, rheumatoid factor, or sIL-2R in spite of complete regression of the ocular lesions after radiotherapy. After administration of cyclophosphamide/doxorubicin/vincris, tine/prednisone and/or rituximab to three patients, all three showed improved serum levels of IgG, rheumatoid factor, and sIL-2CONCLUSIONS: Patients with ocular adnexal MALT lymphoma and polyclonal hypergammaglobulinemia have elevated serum levels of rheumatoid factor, sIL-2R, and IgE, and high dissemination or lymphadenopathy. These unique characteristics may correlate with the systemic immunologic imbalances.