摘要
目的探讨眼附属器滤泡性淋巴瘤(OAFL)的临床病理学特征。方法回顾性病例系列研究。收集1990年1月至2022年5月在天津市眼科医院确诊的10例OAFL患者的临床资料,分析患者的一般资料和病史、发病部位、影像学、组织病理学及分子检测等资料。其中7例进行爱泼斯坦-巴尔病毒(EBV)小编码RNA(EBER)及B细胞淋巴瘤蛋白2(BCL-2)/免疫球蛋白重链基因(IgH)易位基因检测。对患者的治疗和预后进行随访。结果10例(10只眼)患者均为单眼发病,其中男性5例,女性5例,年龄为58(43,68)岁。患者临床表现为眼睑肿胀,结膜增厚呈粉红色,泪腺区、眶内或泪囊区缓慢生长的无痛性肿块。病变位于泪腺4例,眶内肌锥外2例,结膜2例,泪囊1例,泪囊和结膜1例。其中原发性8例,继发性2例。Ann Arbor分期Ⅰ~ⅡE期8例,ⅢE期2例。病理分级1~2级6例,3A级3例;另外1例分别为泪囊区3B级和结膜1~2级。滤泡为主型4例,弥漫型3例,混合型2例;另外1例泪囊区和结膜均为混合型。所有患者肿瘤细胞阳性表达白细胞分化抗原分化群(CD)20、CD21和CD23;9例阳性表达CD10,1例部分细胞阳性表达CD10;所有患者阳性表达B细胞淋巴瘤蛋白6(BCL-6);9例阳性表达BCL-2;特异性周期蛋白-D1(CyclinD1)及多发性骨髓瘤癌基因蛋白1(MUM-1)均阴性表达;ki-67增殖指数为10%~90%。共7例患者进行分子检测,EBER原位杂交未见阳性患者,其中5例存在BCL2与/IgH基因融合。获得随访的7例患者随访时间为17(6,34)个月,其中完全缓解4例,部分缓解2例,因肺部感染死亡1例。结论OAFL是滤泡中心B细胞发生的肿瘤,肿瘤性滤泡阳性表达BCL-2、CD10和BCL-6,可累及泪腺、眶内、泪囊及结膜,患者预后普遍较好。
Objective To investigate the clinical pathological characteristics of ocular adnexal follicular lymphoma(OAFL).Methods A retrospective case series study was conducted.Clinical data of 10 OAFL patients diagnosed at Tianjin Eye Hospital from January 1990 to May 2022 were collected.The study analyzed general patient information,medical history,site of involvement,imaging,histopathology,and molecular detection.Among them,7 cases underwent Epstein-Barr virus-encoded small RNA(EBER)and B-cell lymphoma protein 2(BCL-2)/immunoglobulin heavy chain gene(IgH)translocation gene detection.Treatment and prognosis of patients were followed up.Results All 10 patients(10 eyes)had unilateral involvement,including 5 males and 5 females,with an age range of 58(43,68)years.Clinical manifestations included eyelid swelling,pink conjunctival thickening,painless slow-growing masses in the lacrimal gland area,extraconal muscle cone,conjunctiva,lacrimal sac,or a combination of lacrimal sac and conjunctiva.Among them,8 cases were primary,and 2 cases were secondary.According to the Ann Arbor staging,8 cases were stageⅠ-ⅡE,and 2 cases were stageⅢE.Histopathological grading revealed 6 cases of grade 1-2 and 3 cases of grade 3A.One case showed grade 3B in the lacrimal sac area and grade 1-2 in the conjunctiva.The predominant subtype was follicular in 4 cases,diffuse in 3 cases,and mixed in 2 cases.One case had a mixed subtype involving the lacrimal sac and conjunctiva.All patients expressed positivity for leukocyte differentiation antigens(CD)20,CD21,and CD23.Nine cases were positive for CD10,with 1 case showing partial CD10 positivity.All patients were positive for B-cell lymphoma protein 6(BCL-6),and 9 cases were positive for BCL-2.Specific markers CyclinD1 and Multiple Myeloma Oncogene Protein 1(MUM-1)were negatively expressed in all cases.The Ki-67 proliferation index ranged from 10%to 90%.Molecular detection was performed in 7 patients,with none showing positive EBER in situ hybridization.However,5 cases exhibited BCL2/IgH gene fusio
作者
李静
王玉川
林锦镛
Li Jing;Wang Yuchuan;Lin Jinyong(Tianjin Eye Hospital,Nankai University Affiliated Eye Hospital,Clinical College of Ophthalmology of Tianjin Medical University,Tianjin Eye Institute,Tianjin Key Laboratory of Ophthalmology and Visual Science,Tianjin 300020,China)
出处
《中华眼科杂志》
CAS
CSCD
北大核心
2023年第11期930-936,共7页
Chinese Journal of Ophthalmology
基金
天津市医学重点学科(专科)建设项目(TJYXZDXK-016A)。
