眼附属器黏膜相关淋巴样组织结外边缘区B细胞淋巴瘤21例临床特征及疗效分析

The clinical features and outcomes in 21 patients with ocular adnexal mucosa-associated lymphoid tissue lymphoma

目的分析眼附属器黏膜相关淋巴样组织（MALT）结外边缘区B细胞淋巴瘤（OAML）的临床特征、治疗方法及其预后情况。方法对北京同仁医院2008年6月至2011年12月收治的21例OAML患者的临床资料进行回顾性分析。结果21例患者中，男12例（57．1％），女9例（42．9％），中位年龄57（23～79）岁，大部分为局限性病变，IE期16例（76．2％），眼外播散性病变ⅣE期5例（23．8％）。13例（61．9％）患者肿块完整切除，同时手术后正电子发射计算机体层成像CT（PET．CT）未见代谢活性增高病灶，未给予治疗，中位随访14（5～38）个月后，所有患者仍处于完全缓解（CR）状态。8例（38．1％）患者接受化疗，3例IE期患者采用COP（环磷酰胺＋长春新碱＋泼尼松）或CHOP（环磷酰胺＋阿霉素＋长春新碱＋泼尼松）方案，均获部分缓解（PR）；5例IVE期患者采用COP或CHOP联合利妥昔单抗的方案，均获得CR。全体患者的3年总生存率为100．0％，无病生存率为74．9％。结论OAML大部分为局限性病变，呈惰性临床过程，淋巴瘤相关致死率低，手术切除是重要的治疗方式，对于晚期播散性病变应进行全身化疗，化疗联合利妥昔单抗可以提高缓解率。

Objective To evaluate the clinical features, therapies and prognosis in patients with mucosa-associated lymphoid tissue （MALT） lymphoma in ocular adnexal marginal zone （OAML）. Methods A retrospective analysis was made upon clinical data from 21 patients with OAML admitted into Beijing Tongren Hospital from June, 2008 to December, 2011. Results There were 12 （57.1%） men and 9 （42. 9% ） women, with a median age of 57 （23-79） years old. Majority of patients had localized pathological changes. Among them, 16 patients （76. 2% ） were in stage I E, and 5 （23.8%） in stage 1VE. Surgical resection as the sole treatment was performed in 13 patients （61.9%）, and positron emission tomography CT（PET-CT） imaging demonstrated normal fluorine 18-fluorodeoxyglueose （FDG） uptake after surgical resection, who were managed with no further therapy. All the 13 patients were followed up for median 14 （5-38） months, and all in complete remission. Combination chemotherapy was given to 8 （38. 1% ） patients. Three patients in stage I E treated with COP （cyelophosphamide, vincristine and prednisone） or CHOP （eyclophosphamide, adriamycin, vincristine and prednisone ） were all in partial remission. Five patients in stage IVE were treated with COP/CHOP in combination with rituximab, and all in complete remission. The 3-year overall survival rate and disease-free survival rate in the total patients were 100. 0% and 74. 9% respectively. Conclusions The patients with OAML generally have localized disease, show indolent clinical course, and present low lymphoma-related mortality. Surgical resection is a very important treatment in the patients with local disease. Systemic chemotherapy should be considered in patients at advanced stages. Rituximab in combination with chemotherapy can improve the remission rate.