目的：探讨眼眶黏膜相关淋巴组织(mucosa-associated lymphoid tissue，MALT)结外边缘区B细胞淋巴瘤的临床表现、影像学特征及病理学特点，以期减少眼眶淋巴瘤的误诊，提高生存率。方法：对2020年1月至2020年12月中山大学中山眼科中心诊治的71例眼眶MALT结外边缘区B细胞淋巴瘤的临床、影像及病理学资料进行回顾性分析。结果：71例患者中，男38例，女33例；左侧眼眶31例，右侧眼眶34例，双侧眼眶6例；原发病例67例，复发病例4例；年龄23~84岁，病程为3个月~15年。最常见的临床表现是眼部肿块和不同程度的眼球活动受限；磁共振成像(magnetic resonance imaging，MRI)检查见密度均匀的软组织影，呈“铸造样”，眼球内未见侵犯；组织病理检查可见肿瘤由小至中等大小淋巴样细胞组成，瘤细胞呈弥漫或片状生长，核小到中等、不规则，核仁不明显，部分细胞呈单核样淋巴瘤细胞改变，其中9例可见浆细胞样分化，伴浆细胞分化的病例kappa与lambda的表达不对称。结论：眼眶MALT结外边缘区B细胞淋巴瘤有多种临床表现，影像学检查具有一定的特征，可辅助术前诊断。病理学检查可用于术后的准确诊断及分型，据此制定合适的治疗方案，提高疗效。
Objective: To investigate the clinical, imaging and pathological features of orbital extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT), so as to reduce misdiagnosis and improve survival rate.Methods: The clinical, imaging and histopathological data of 71 patients with orbital extranodal marginal zone B cell lymphoma of MALT who were treated in Zhongshan Ophthalmic Center, Sun Yat-sen University from Jan.2020 to Dec. 2020 were retrospectively analyzed. Results: There were 38 males and 33 females in the 71 patients.There were 31 cases located on the left orbit, 34 cases on the right orbit, and 6 cases were bilateral; there were 67 primary cases and 4 relapsed cases. The age ranged from 23 to 84 years old. The disease course ranged from 3 months to 15 years. The main clinical manifestations included ocular masses and limited eyeball movement.Magnetic resonance imaging showed a “cast-like” soft tissue shadow of uniform density, and no intraocular invasion is observed. Histopathological examination showed that the tumor was composed of small to medium sized lymphocyte-like cells. Tumor cells exhibited diffuse or lamellar growth, with small to medium-sized irregular nuclei, and the nucleoli were inconspicuous. Some of the lymphocyte-like cells showed mononuclear lymphoma cells changes, and plasmacytoid differentiation was observed in 9 cases, in which kappa and lambda expressions were asymmetric.Conclusion: The clinical manifestations of orbital extranodal marginal zone B cell lymphoma of MALT are various, and imaging examination has certain characteristics, which can assist preoperative diagnosis.Pathological examination can be used for accurate diagnosis and classification after operation, based on which appropriate treatment plan can be made and curative effect can be improved.
黏膜相关淋巴组织(mucosa-associated lymphoid tissue lymphomas，MALT)淋巴瘤是原发性眼附属器淋巴瘤(primary ocular adnexal lymphoma，POAL)中最常见的病理类型。目前，原发性眼附属器黏膜相关淋巴组织淋巴瘤(primary ocular adnexal mucosa-associated lymphoid tissue lymphoma，POAML)的临床类型和临床表现尚未被眼科医师熟练掌握，临床治疗亦无共识和指南。本文根据POAML起源位置，重点介绍各临床类型的早中期临床表现，以及针对各临床类型和病变范围的个体化治疗方法。
Mucosa-associated lymphoid tissue lymphomas (MALT) lymphoma is the most common pathologic type in primary ocular adnexal lymphoma (POAL). Currently, the clinical types and manifestations of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) have not been well understood by ophthalmologists, and there is no consensus or guideline for clinical treatment. According to the original location, this paper focuses on the clinical manifestations of early and intermediate-stage POAML, as well as the individualized treatment for each clinical type and lesion range.
近年来日益强大的眼内液分子/细胞生物学检测技术因其简便、快捷和高效的特点，使得眼科医生在诊断眼内淋巴瘤时倾向于单纯只依据此类方法而淡化病理诊断的重要性。眼内液分子/细胞生物学技术因其本身只能“间接提示肿瘤细胞存在”的局限性而不能作为眼内淋巴瘤的确诊依据。眼内组织/细胞病理仍然是眼内淋巴瘤诊断的金标准，其价值和地位不能被其他任何分子/细胞生物学检测手段所替代。理解并掌握各种诊断、检测技术的优势和局限性，规范和优化眼内组织/细胞病理标本的采集、保存和送检流程有助于提高眼科临床医生对眼内淋巴瘤的诊断效率和医疗质量。
In recent years, more and more powerful molecular/cellular biological techniques of intraocular fluid have made ophthalmologists tend to only rely on these methods in the diagnosis of intraocular lymphoma because of their features of simplicity, fastness and efficiency. The molecular/cellular biological techniques of intraocular fluid cannot be used as the basis for the diagnosis of intraocular lymphoma because it can only indicate the existence of tumor cells indirectly. Intraocular tissue/cell pathology remains the gold standard for the diagnosis of intraocular lymphoma, and its importance cannot be replaced by any other molecular/cell biological methods. Understanding and mastering the advantages and limitations of various diagnostic techniques, standardizing and optimizing the collection, preservation and submission process of intraocular tissue/cell specimens will help ophthalmologists improve the diagnostic efficiency and medical quality of intraocular lymphoma.
眼附属器淋巴组织增生性疾病作为一类疾病的总称，包括了良性淋巴组织增生、非典型性淋巴组织增生、IgG4相关眼病以及多种恶性淋巴瘤在内的数十种疾病类型。临床诊断此类疾病应将患者眼部体征、影像学检查与病理学检查紧密结合。随着免疫表型及分子病理等检测技术的进步，此类疾病之间的鉴别诊断正逐渐清晰。本文就眼附属器淋巴组织增生性疾病进行系统性描述，并重点探讨该类疾病的病理鉴别诊断。
Ocular adnexal lymphoproliferative disease, as a general term, contains reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, IgG4 related ocular disease and malignant lymphoma. The clinical diagnosis of this kind of disease should integrate patient’s symptoms, imaging features and pathology characteristics. Development of immunophenotyping, molecular pathology and other detection technology will help with the differential diagnosis of ocular adnexal lymphoproliferative disease. This article is going to discuss the etiology, epidemiology,diagnosis and treatment of ocular adnexal lymphoproliferative disease, with a focus on the clinicopathological differential diagnosis of such disease.
眼内淋巴瘤(intraocular lymphoma，IOL)比较罕见。按起源位置分为两种类型，主要类型为原发性眼内淋巴瘤(primary intraocular lymphoma，PIOL)，也称为原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma，PCNSL)；另外一种类型为继发性眼内淋巴瘤(secondary intraocular lymphoma，SIOL)，为中枢神经系统以外的淋巴瘤转移至眼内。按肿瘤类型主要分为三类，主要类型为眼内弥漫大B细胞淋巴瘤，属于高级别淋巴瘤，预后较差；其次为少见的主要侵犯脉络膜的黏膜相关淋巴组织结外边缘区B细胞淋巴瘤，属于低级别淋巴瘤，预后较好；第三种类型为极少见的眼内NK/T细胞淋巴瘤，属于高级别淋巴瘤，预后极差。该病的诊断对眼科医生和病理医生都极具挑战性。实验室检测方法主要包括病理学、免疫细胞化学、流式细胞术、细胞因子及基因重排等，但眼内病理活检仍然是该病诊断的金标准。该病的治疗主要为眼内局部化疗、放射治疗及系统性化疗。IOL早期常因误诊而耽误治疗，目前该病明确诊断时多在患者出现症状后4~40个月，多数病例早期被误诊为葡萄膜炎而失去治疗的最佳时机，导致预后较差。因此应充分认识IOL的早期表现，早期诊断、早期治疗，从而大大提高疗效。
Intraocular lymphomas (IOL) are rare malignant neoplasms including primary intraocular lymphoma (PIOL) and secondary intraocular lymphoma (SIOL). The former is also known as primary central nervous system lymphoma(PCNSL). The latter is a kind of lymphoma metastasizing to the eye from outside the central nervous system. IOL can further be divided into three different types. The most common type is vitreoretinal high-grade diffuse large B-cell lymphoma with poor prognosis. The less common type is primary choroidal extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue, which is low-grade B-cell lymphoma with better prognosis.The rare type is NK/T cell lymphomas with very poor prognosis. The diagnosis of this disease is challenging for both ophthalmologists and pathologists. Laboratory testing methods mainly include cytology/pathology,immunocytochemistry, flow cytometry, cytokine analysis and gene rearrangement detection. The detection of malignant lymphoid cells cytologically/pathologically is still the gold standard for diagnosing IOL. The treatment involves local chemotherapy, radiotherapy and systemic chemotherapy. Most intraocular lymphomas at early stage are misdiagnosed as uveitis and proper treatment is often delayed with poor diagnosis due to the lost of best time for treatment. So far, the delay between the diagnosis and the onset of ocular symptoms ranges from 4 to 40 months. Therefore, we should fully understand the early manifestations of intraocular lymphoma and early diagnose and timely treat the disease in order to improve prognosis.
白内障是世界范围内失明的主要原因。正常晶状体是富有弹性的形似双凸透镜的透明体，是机体内蛋白质含量最高的组织，由晶状体囊膜、晶状体上皮细胞、晶状体纤维和悬韧带构成。白内障为晶状体透明度下降，表现为晶状体混浊。近年来随着分子生物学、表观遗传学、免疫学、有机化学等学科快速发展，国内外学者对白内障也进行了大量分子水平的研究，探讨了白内障发生发展相关分子机制，为未来基因治疗和靶向药物等治疗白内障提供了理论基础。对白内障分子病理改变的了解，是白内障精准诊治的基础。
Cataract is the main cause of blindness worldwide. The normal crystalline lens is a transparent biconvex disc,with highest protein content in all human tissues. The lens is composed of capsule, lens epithelial cells, lens fiber and zonular ligment. Cataract is a decrease in the transparency of the lens, which is characterized by opacity. In recent years, with the rapid development of molecular biology, epigenetics, immunology and organic chemistry,researchers have conducted a large number of studies on the molecular basis of genetic or targeted therapy of cataract. It is important to know the molecular pathology of cataract, which is the basis of precise diagnosis and treatment of cataract.
线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。
Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.
眼眶部副神经节瘤极为罕见，多属非功能性肿瘤。本病例为1位中年女性，以右眼眼睑抬举无力为主诉就诊，眼眶计算机断层扫描(computed tomography，CT)和磁共振成像(magnetic resonance imaging，MRI)检查发现右眼眼眶内肿物，手术完整切除，结合HE染色和免疫组织化学检查，病理组织学诊断为眼眶副神经节瘤，随访3年，肿瘤无复发。
Orbital paraganglioma is a rare disease and mostly belongs to non-functional tumors. In this report, we described a middle-aged female admitted to our hospital with the chief complaint of weak lifting of her right eyelid. Orbital computed tomography (CT) and magnetic resonance imaging (MRI) examination detected an intra-orbital mass in the right eye. Complete excision was conducted, and post-operative histopathological and immunohistochemical examination revealed the mass to be orbital paraganglioma. Within the 3-year follow-up,the patient showed no recurrence.
上皮内生是眼外伤或眼前节手术后罕见的严重并发症，可导致角膜内皮失代偿、继发性青光眼或其他不良结果。其中难治性青光眼是上皮内生后眼球摘除的主要原因，因此提高对本病的认识并严加防范至关重要。本文回顾分析1例上皮内生性青光眼患者的临床资料和病理切片，结合文献讨论本病的危险因素、发病机制及防范措施。
Epithelial downgrowth is a rare yet serious complication after ocular trauma or anteriorsegmental surgery. It can lead to decompensation of corneal endothelium, secondary glaucoma or other serious complications, among which refractory glaucoma is the main cause of enucleation. It is vital to raise the awareness of this disease and take strict precautions against it. We present a case of epithelial downgrowth and discuss the risk factors, pathogenesis and preventive measures of the disease through analyzing clinical data and pathological sections.
目的：比较重力液流与主控液流2种灌注方式下行白内障超声乳化手术对青光眼患者视盘血流的影响。方法：采用随机数字表法将患者分为2组，分别为重力液流灌注组和主控液流灌注组。记录术中超声乳化累积释放能量(cumulative dissipated energy，CDE)，术后1天、1周、1个月和3个月患者最佳矫正视力(best corrected visual acuity，BCVA)、眼压、视盘血流密度及视网膜神经纤维层厚度。结果：主控液流灌注组术中CDE小于重力液流灌注组(5.6±1.3 vs 6.3±1.2，P=0.034)。术后1天重力液流灌注组视盘周围血管密度(circumpapillary vascular density，cpVD)、整个图像血管密度(whole en face image vessel density，wiVD)和视盘内血管密度(inside disc vascular density，inside disc VD)均高于主控液流灌注组(P<0.05)，其余时间点差异无统计学意义(P>0.05)。术后1周和1个月重力液流灌注组视网膜神经纤维层厚度大于主控液流灌注组(P<0.05)，术后1天和3个月未见明显差异。结论：相较于传统的重力液流灌注，主控灌注能够在青光眼患者白内障超声乳化手术中减少超声能量的使用，术后早期可减轻由术中高眼压引起的视盘炎症性充血，可以减轻对视网膜神经纤维层的影响。
Objective: To compare the influence of active versus passive phacoemulsification fluidics systems on optic disc blood flow in patients with glaucoma. Methods: Patients were divided into 2 groups by a random number table method, namely the active fluidics system group and the passive fluidics system group. The intraoperative cumulative dissipated energy (CDE) was recorded, and the best corrected visual acuity (BCVA), intraocular pressure, optic disc blood flow density and retinal nerve fiber layer thickness were measured at the follow-up of 1 day, 1 week, 1 month and 3 months. Results: During phacoemulsification, CDE in the active fluidics system group was lower than that in the passive fluidics system group (5.6±1.3 vs. 6.3±1.2, P=0.034). One day after the surgery,the circumpapillary vessel density (cpVD), whole image vessel density (wiVD) and inside disc vascular density(inside disc VD) in the passive fluidics system group were higher than those in the active fluidics system group(P<0.05), and the differences were not statistically significant at the rest of the follow-ups (P>0.05).The retinal nerve fiber layer in passive fluidics system group was thicker than that in active fluidics system group at the follow-ups of 1 week and 1 month (P<0.05), and the difference was not statistically significant at the follow-up ofs 1 day and 3 months. Conclusion: Compared with the traditional passive fluidics system, the active fluidics system can reduce the CDE during phacoemulsification surgery. It can reduce the inflammatory congestion of the optic disc caused by intraoperative high intraocular pressure on the early postoperative stage. In addition, it can also protect retinal nerve fiber layer.
