原发性眼部淋巴瘤的临床分析及治疗预后

发布时间：2018-09-10 19:47

【摘要】：目的:眼部淋巴瘤发病率低、初期诊断困难,本研究针对眼部淋巴瘤的起病受累部位、临床特点、病理分型、诊断及治疗方案、预后进行评估,尤其针对眼附属器MALT淋巴瘤患者的预后因素进行分析,为标准化方案的制定提供临床资料。方法:收集我院自2000年1月至2016年12月经眼部活组织检查及免疫组化确诊的眼部淋巴瘤患者,其中包括原发性眼内淋巴瘤患者共10例,眼附属器淋巴瘤患者共86例,对比患者临床特点、早期表现、受累部位、病理分型、治疗方法、预后随访评估。病例的临床分期应用Ann-Arbor法,治疗疗效的评估按照IWG评价标准,数据分析应用SPSS软件,Kaplan-Meier曲线计算生存率,并用Log-rank法进行检验;定性资料的分析采用卡方和Fisher确切概率检验。结果:共收集原发性眼部淋巴瘤96例,原发性眼附属器淋巴瘤(POALs)的86例患者中,眼眶受累者64例(74.42%),结膜受累者21例(24.42%),泪腺受累者1例(1.16%)。病理分型以B细胞来源为主(84例),其中MALT淋巴瘤最为常见,共78例(90.70%)。来诊患者临床表现不典型,但多由于肿瘤所导致的占位表现。影响POALs预后的因素包括病理类型、治疗方案,而发病部位不作为影响预后的危险因素。Ann-Arbor分期I期的MALT淋巴瘤患者缓解率和5年无进展生存率高,单纯手术治疗组5年无进展生存率较放疗组及手术联合放疗组生存率低。原发性眼内淋巴瘤(PIOL)患者共10例,表现为玻璃体受累者8例,以单眼受累居多。其主要临床表现为眼部异物感、畏光、视物模糊。所有患者的病理学分型均为弥漫大B细胞淋巴瘤,单眼受累的7名患者给予局部治疗(玻璃体腔注射剂联合局部放疗),均获得完全缓解,其中3例患者复发,补救治疗效果欠佳,2例死亡,1例患者随访3个月无复发;2例双眼同时受累的患者应用局部治疗(玻璃体腔注射+局部放疗)联合全身化疗,随访12-22个月未复发;双眼受累合并神经系统症状的1例老年患者给予利妥昔单抗未获得缓解,经治无效死亡。结论:1)在原发性眼部淋巴瘤患者中,POALs所占比例远高于PIOL,其中眼眶为最常见的好发部位,其次为结膜。PIOL好发于玻璃体。2)POALs患者中最常见的病理类型为MALT淋巴瘤,其次为滤泡性淋巴瘤;PIOL患者最常见的病理学类型为弥漫大B细胞淋巴瘤。3)POALs患者的预后较为理想,影响因素主要包括病理分型、治疗方案,其中以MALT淋巴瘤的预后最为理想;IE期的MALT淋巴瘤患者单纯手术治疗更易出现远期复发或进展,而局部放疗或放疗联合手术治疗方案则具有较高的5年无进展生存率。4)原发性眼内淋巴瘤的恶性程度高、侵袭性大,无共识性一线治疗方案,治疗方案的选择需根据受累部位评估。
[Abstract]:Objective: the incidence of ocular lymphoma is low and the initial diagnosis is difficult. In this study, the location, clinical features, pathological classification, diagnosis and treatment, prognosis of ocular lymphoma were evaluated. The prognostic factors of ocular adnexal MALT lymphoma were analyzed in order to provide clinical data for the establishment of standardized protocol. Methods: from January 2000 to December 2016, 10 cases of ocular lymphoma were diagnosed by ocular biopsy and immunohistochemistry, including 10 cases of primary intraocular lymphoma and 86 cases of ocular adnexal lymphoma. The clinical features, early manifestations, site of involvement, pathological classification, treatment, and prognosis were compared. Ann-Arbor method was used to evaluate the therapeutic effect according to IWG evaluation standard. The survival rate was calculated by SPSS software Kaplan-Meier curve and tested by Log-rank method. The qualitative data were analyzed by chi-square and Fisher exact probability test. Results: among 96 cases of primary ocular lymphoma and 86 cases of primary ocular adnexal lymphoma, 64 cases (74.42%) were involved in orbit, 21 cases (24.42%) were involved in conjunctiva, 1 case (1.16%) was involved in lacrimal gland. The pathological classification was mainly B cell origin (84 cases), of which MALT lymphoma was the most common, 78 cases (90.70%). The clinical manifestations of these patients are not typical, but most of them are caused by tumors. The factors influencing the prognosis of POALs included pathological type, treatment regimen, and the location of the disease was not a risk factor for prognosis. The remission rate and 5-year progression-free survival rate of patients with stage I MALT lymphoma in Ann-Arbor stage were high. The 5-year progression-free survival rate in the simple operation group was lower than that in the radiotherapy group and the surgery combined radiotherapy group. There were 10 cases of primary intraocular lymphoma (PIOL) with vitreous involvement in 8 cases, most of which were monocular involvement. The main clinical manifestations are ocular foreign body sensation, photophobia and blurred vision. All patients were classified as diffuse large B-cell lymphoma, 7 patients with monocular involvement were treated with local therapy (vitreous injection combined with local radiotherapy), and 3 of them relapsed. There were 2 patients who died after 3 months follow up and 2 patients with bilateral involvement were treated with local therapy (local intravitreal injection radiotherapy) combined with systemic chemotherapy, followed up for 12 to 22 months without recurrence. One elderly patient with binocular involvement and neurological symptoms received rituximab without remission and died after treatment. Conclusion (1) the percentage of POALs patients with primary ocular lymphoma is much higher than that with PIOL,. The most common location is orbital, followed by conjunctival. PIOL. 2) MALT lymphoma is the most common pathological type in POALs patients. Secondly, the most common pathological type of follicular lymphoma was diffuse large B-cell lymphoma (3. 3) the prognosis of POALs patients was satisfactory. The main influencing factors included pathological classification and treatment plan. Among them, MALT lymphoma patients with MALT lymphoma had the most ideal prognosis in IE stage and were more likely to have long term recurrence or progression after simple surgical treatment. Local radiotherapy or radiotherapy combined with surgery had a higher 5-year progression-free survival rate (.4) primary intraocular lymphoma had a higher degree of malignancy, greater invasiveness, and no consensus first-line treatment. Treatment options need to be evaluated according to the site involved.
【学位授予单位】：青岛大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R739.7

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