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.
目的:分析泪阜部色素痣合并鳞状细胞乳头状瘤的临床及组织病理学特征。方法:选取2002年1月至2020年6月天津市眼科医院经手术切除的色素痣合并鳞状细胞乳头状瘤1 0例,分析患者的临床及组织病理学特征。结果:10例患者中男性5例(50%),年龄(50.20±19.57)岁,左眼5例,右眼5例,其中7例(70%)为复合痣,3例(30%)皮内痣,未见交界痣,7例复合痣和2例皮内痣可见结膜上皮细胞囊肿;鳞状细胞乳头状瘤中,有蒂型7例(70%),无蒂型3例(30%),9例(90%)含结膜杯状细胞,1例(10%)伴有鳞状上皮角化,3例(30%)伴慢性炎症,2例(20%)伴有轻度非典型增生,所有病例未见明显的弹力纤维变性。结论:泪阜部色素痣合并鳞状细胞乳头状瘤比较少见,外观易误诊为色素痣恶变或黑色素瘤,临床上应注意甄别,确诊有赖于组织病理学检查。
Objective: To analyze the clinical pathologic characteristics of pigment nevus of lacrimal caruncle complicated with squamous cell papilloma.Methods: This is retrospective case series study including ten patients diagnosed as pigment nevus of lacrimal caruncle complicated with squamous cell papilloma who underwent surgical treatment at Tianjin Eye Hospital from January 2002 to June 2020. The clinical and histological data were abstained for statistical analysis. Results: Among the included 10 patients 5 (50%) were males. The mean age was 50.20±19.57 years (ranged, 21 to 72 years). Five cases involved with the right eye and the other 5 cases involved with the left eye. In term of subtype of nevus, 7 cases (70%) were compound nevus and the left 3 cases (30%) were intradermal nevus. The conjunctival epithelial cysts were found in 7 cases of compound nevus and 2 cases of intradermal nevus. Regarding the clinical appearance of papilloma, 7 cases were pedunculated and 3 cases were sessile. There were 9 papilloma eyes containing conjunctival goblet cells, 1 shows hyperkeratosis, 3 with chronic inflammation,2 showed mild atypia. There was no obvious elastosis in any of the tumors.Conclusion: The pigment nevus of lacrimal caruncle complicated with squamous cell papilloma mainly localized were not common. They were easily misdiagnosed as melanoma and the diagnosis depended on histopathological examination.
目的:探讨眼囊尾蚴病的临床病理学特点。方法:回顾性分析7例眼囊尾蚴病的临床资料、大体与镜下病理改变。结果:男女性别比1:6,平均年龄24.7(中位数20)岁。临床以眼睑与眼周红肿、肿胀为主要症状3例,视物不清、视力下降2例,复视1例,结膜红肿1例。囊尾蚴寄生于球结膜1例,寄生于眼球内2例,寄生于眼眶内4例。7例均见到猪囊尾蚴,均在虫体内找到石灰小体,其中2例伴有坏死,可在坏死物中找到石灰小体。结论:眼囊尾蚴病以青少年女性多见,眼内型以视力下降为主要症状,眼眶型以眼睑与眼周红肿、肿胀为主要症状。找到囊尾蚴虫体与头节是眼囊尾蚴病明确诊断依据之一,石灰小体是该病重要病理诊断线索。
Objective:To explore the clinicopathological characteristics of ocular cysticercosis. Methods: The clinical data,grossing and microscopic pathological changes of 7 cases of ocular cysticercosis were analyzed retrospectively.Results: In this study, the male-to-female ratio was 1:6, the mean age was 24.7 (median age 20) years old. Of these patients, 3 patients presented with eyelid and periocular redness and swelling, 2 with blurred vision and decreased vision, 1 with diplopia, and 1 with conjunctival swelling and redness. In addition, 1 case was parasitized by cysticercus in the bulbar conjunctiva, 2 cases were in the eyeball and 4 cases were in the orbit. Cysticercus cellulosae containing calcareous corpuscle were observed in all cases, including 2 were accompanied by necroses with calcareous corpuscle.Conclusion: Ocular cysticercosis is found to be more common in female adolescents.Decreased vision is the main clinical symptom in intraocular infections, while orbital infections often show redness and swelling of eyelid and periocular. The finding of cysticercus and scolex in specimens is one of the confirmed diagnostic bases of ocular cysticercosis and calcareous corpuscles are important pathological diagnostic clues for this disease.
目的:制备抗水通道蛋白4(aquaporin 4,AQP4)单克隆抗体,鉴定其免疫学性能为后期临床应用奠定基础。方法:合成AQP4优势抗原表位多肽,经过免疫小鼠、细胞融合及亚克隆筛选,制备可分泌高效价高亲和力的单克隆抗体细胞株,后期获取大量单克隆抗体通过酶联免疫吸附和免疫组织化学实验进行初步实验应用。结果:获得4株杂交瘤细胞株,均能稳定分泌高亲和力抗体,经酶联免疫吸附测定实验和免疫组织化学实验证实均能特异性识别人的AQP4蛋白。结论:成功制备了亲和力高、特异性强的抗人AQP4优势抗原表位的单克隆抗体,为小分子抗体以及抗体人源化制备奠定了基础,进而为治疗视神经脊髓炎相关疾病提供理论依据和技术支持。
Objective: To lay the foundation for clinical applications in the future, we prepare and identify the immunological properties of aquaporin 4 (AQP4) monoclonal antibodies. Methods: The dominant epitope polypeptides of AQP4 were synthesized and used to obtain the cell lines which secrete high tiler and high affinity monoclonal antibodies by immunizing BALB/c mouse cell fusion and screening of subclone. The large amount of monoclonal antibodies were obtained and used for practice via immunohistochemical staining and Enzyme-linked immunosorbent assay (ELISA). Results: Four hybridomas that can stably secret anti-AQP4 antibodies were obtained. ELISA, Western blot and Immunohistochemistry results suggested that the monoclonal antibodies specifically recognized human AQP4 protein.Conclusion: The anti-human AQP4 monoclonal antibody with high affinity and specificity was successfully generated, which thereby provides the foundation for the preparation of small molecule antibodies or humanized antibodies, and then lays a theoretical and technical basis for the treatment of neuromyelitis optica (NMO).