儿童横纹肌肉瘤的临床病理研究

目的 探讨横纹肌肉瘤的临床病理、免疫组织化学、电镜特点。方法 对145例患儿(男97例,女48例,年龄4个月～13岁,平均4．2岁),用HE、免疫组织化学(LSAB法)和电镜技术进行观察。所用抗体有：波形蛋白、结蛋白、平滑肌肌动蛋白(SMA)、肌红蛋白、神经元特异性烯醇化酶(NSE)、CD99(12E7)、白细胞共同抗原(LCA)。结果 145例患儿中,100例随访1～20年。组织学分为胚胎性、葡萄状、梭形细胞型、腺泡状和实性型。5年存活率：葡萄状85％、梭形细胞型横纹肌肉瘤85％,胚胎性60％,腺泡状40％、实性型横纹肌肉瘤25％,预后最好的是梭形细胞型和葡萄状横纹肌肉瘤,预后次之的是胚胎性横纹肌肉瘤,预后差的是腺泡状和实性型横纹肌肉瘤;预后好的部位是膀胱和头颈部,预后差的部位是躯干四肢、腹膜后和盆腔;免疫组织化学：107例均表达波形蛋白,除NSE、CD99和LCA均阴性外,结蛋白、SMA和肌红蛋白阳性率分别为78％、75%和37％。15例肿瘤经电镜观察,其中10例发现有肌丝和肌节结构。结论 免疫组织化学和电镜检查有利于儿童横纹肌肉瘤的病理诊断和组织分型。     

人眼结膜杯状细胞的定量研究

目的：研究杯状细胞在结膜各个方位的分布、形态及数量。方法：6眼（30－90岁）结膜,采用切片结合铺片的方法,1％阿利新兰,0．3％甲苯胺兰,HE染色,经显微摄像头输入Macintosh图象处理系统计数。结果：杯状细胞呈多房空泡状,圆形,核偏 位,单个,散在或密集分布。不同年龄组均以鼻下象限数量最多。80－90岁组杯状细胞的绝对数明显下降。结论：切片,铺片联合图象处理系统可以定量,准确地计算杯状细胞在结膜中的绝对数,从而为眼表疾病的研究提供有力的参考指标。     

原子力显微镜应用于红细胞病理学检验的初步研究

目的: 初步探讨原子力显微镜在临床病理学检验中的应用前景。方法: 应用原子力显微镜对正常人、肺癌病人和骨髓增生异常综合症等病人血液中的红细胞进行大范围和微观结构的形貌图像与数据的获取和分析。结果: 用原子力显微镜获得了多个红细胞、单个红细胞和细胞膜表面微观结构清晰的形貌图像,发现肺癌病人大多数红细胞呈棘刺状,每个细胞共有10-20个棘刺状突起,大部分突起在细胞的边缘,中央有少量突起。细胞边缘的棘刺比中央的棘刺要宽,但较短,平均宽度约为589.0nm,平均长度约为646.7nm。中央棘刺状突起并非伸向细胞片面外,而是倒伏包埋在细胞中。而骨髓增生异常综合症病人的红细胞呈双凹状,膜表面微观结构观察还发现,骨髓增生异常综合症病人的红细胞膜表面出现许多几十nm到一百多nm大小不等的孔洞。结论: 原子力显微镜可广泛地应用于临床病理学检验。其应用前景包括细胞记数,细胞直径、平均高度、体积、表面积和表面积/体积比等参数的获取和比较,单个细胞的形貌观察,以及细胞膜表面微观结构的观察和比较,等等。     

中国流行性出血热脏器标本病毒样颗粒属性的探讨

本文应用电镜观察了12例流行性出血热(EHF)尸体标本,其中7例(58%)出现了泡状病毒样颗粒。它为圆形或椭圆形,直径72～116nm,平均92nm;有包膜,没有核样体,表面粗糙或有微突。颗粒主要分布于网织细胞及心、肝实质细胞的核膜及线粒体,偶尔见于内质网和胞浆。泡状病毒样颗粒在大小和形态上,与洪涛等在培养细胞见到的EHF病毒以及其他学者观察的汉坦病毒和布尼亚病毒基本一致,为EHF病毒在人体脏器细胞的形态表现。     

无标记淋巴细胞

Greenberg等人(1973)首次使用“无标记淋巴细胞”这一概念,用来描述没有T、B标记的淋巴细胞。无标记细胞在人外周血中占2-5.5％,在脑脊髓液中占20～29％;在小鼠脾中为3-13％,而淋巴结为4％以下,在大鼠和小鼠骨髓中含量最高,分别为41％和50％。     

大鼠脾树状突细胞的研究——光镜和电镜观察

本研究采用Kamperdijk的方法,稍加改良,分离了大鼠脾树状突细胞,并对分离的细胞进行了光镜和电镜检测。结果表明细胞95％以上存活。在悬液中,细胞保持突起,且突起能持续伸缩。细胞PAS反应、Prussian blue反应、AlP、PO、ATPase反应均呈阴性,AcP反应或呈阴性,或在核凹陷处呈大圆点状反应,或在核附近有数个小颗粒反应。细胞膜表面有许多Ia抗原,但缺乏Fc受体。细胞表面光滑,突起呈鳞茎状或薄片状。核形不规则。细胞质内细胞器稀少,但可有中等量的线粒体、核糖体和泡状结构。结果表明,分离细胞的形态结构与Steinman和Kamperdijk描述的淋巴树状突细胞类同;分离方法切实可行。     

鼻腔鼻窦腺泡状横纹肌肉瘤10例临床病理分析

目的 探讨鼻腔鼻窦腺泡状横纹肌肉瘤(sinonasal tract alveolar rhabdomyosarcoma,SNT-ARMS)的临床表现、组织学形态、免疫表型及分子遗传学特征、诊断及预后。方法 收集10例SNT-ARMS的临床病理资料,行HE及免疫组化EnVision法染色。应用FISH法检测FOXO1基因断裂重组情况,并复习相关文献。结果 眼观:临床送检为息肉样病变,切面灰白、灰红色,质嫩。镜检:肿瘤由幼稚的小蓝圆细胞组成,呈巢状分布,瘤巢之间可见纤细的纤维血管间隔,局灶瘤巢中央的瘤细胞变性、脱落,形成特征性的腺泡状结构;部分病例肿瘤细胞呈弥漫实性片状分布。肿瘤细胞形态一致,核染色质粗,可见细小核仁,核分裂活跃;肿瘤胞质分化不明显,少数病例部分细胞含有丰富的透明状胞质。部分病例肿瘤内可见数量不等的花环状多核巨细胞及横纹肌母细胞分化。免疫表型:表达横纹肌免疫组化标志物desmin、Myogenin和(或)MyoD1,部分病例表达CKpan、CK-L、Syn、CD56、ALKp80,不表达CK5/6、CgA、NSE、NeuN、NF、S-100、CD99、CD34、CD20、CD3、Tdt等,Ki-67增殖指数为50%～80%。FISH法检测均显示FOXO1基因断裂重组。结论 SNT-ARMS需与其它类型的小蓝圆细胞肿瘤进行鉴别,部分肿瘤表达上皮、神经内分泌标志物,易误诊为低分化癌或神经内分泌癌。     

腺泡状软组织肉瘤的临床病理分析

目的：观察腺泡状软组织肉瘤的临床及病理形态学特点,并探讨其组织发生方法：对12例腺泡状软组织肉瘤进行光镜观察,并对全部病例做组化及免疫组化染色。结果：12例腺泡状软组织肉瘤,男性4例,女性8例,发病年龄16－38岁（平均24岁）。发生部位主要位于四肢尤其是下肢的深部软组织,与骨髓肌关系密切。镜下瘤细胞呈胞泡状排列,周围为富含血窦的间质,瘤细胞质内含有丰富的嗜酸性颗粒,PAS全部阳性,免疫组化：SMA阳性率75％,desmin阳性率66．6％,myoglobin阳性率25％。结论：支持腺泡状软组织肉瘤的组织发生为横纹肌来源或向横纹肌分化。     

湖南长沙及湘西泡状带绦虫分离株线粒体cox1基因的克隆及序列分析

目的研究湖南省长沙及湘西泡状带绦虫分离株线粒体细胞色素C氧化酶第1亚基（cox1）基因部分序列（pcox1）的遗传变异情况,并用peox1序列重构泡状带绦虫与其他带科绦虫的种群遗传关系。方法应用聚合酶链反应（PCR）扩增泡状带绦虫虫株的pcox1,应用Clusta1X1．81程序对序列进行比对。然后用Phylip3．67程序MP法和Mage4．0程序NJ法绘制种系发育树,并用Puzzle5．2程序构建最大似然树;同时利用DNAstar5．0中的Megalign程序进行同源性分析,并与GenBank^TM中已知泡状带绦虫相应基因序列进行比较分析。结果所获得的pcox1序列长度一致,均为343bp,与GenBank^TM公布的带科绦虫序列进行比较分析表明,湖南长沙分离株1和湘西分离株5与已知泡状带绦虫相应基因的相似性分别为99．4％和99．7％,与其它带科绦虫的相似性均小于90．0％;湖南长沙1和湘西分离株5与已知泡状带绦虫相应基因遗传距离分别为0．006和0．003,与其他带科绦虫的遗传距离均大于0．100。种系发育树表明,2个分离株与已知泡状带绦虫位于同一分枝,Bootstrap值在97％以上。结论由于泡状带绦虫pcox1序列种内相对保守,种问差异较大,故可作为种间遗传变异研究的标记,研究结果为进一步研究泡状带绦虫的群体遗传结构奠定了基础。     

脑动脉硬化对外侧膝状体血供影响的形态学研究及临床意义

目的：研究外侧膝状体动脉的来源、数量、分布以及相关动脉的病理变化，为外侧膝状体因缺血所致视野缺损提供形态学依据。方法：在体视显微镜和手术显微镜下，对45例90侧，成人脑标本进行观察，研究外侧膝状体动脉的起源、数量、分布，对其中50～70岁的60侧脑标本的外侧膝状体相关动脉做病理切片观察。结果：外侧膝状体的血供主要来自脉络丛后外动脉、丘脑膝状体动脉和膝络丛前动脉，其中多动脉来源型占80．0％，单一动脉来源型占20．0％。病理组织切片观察发现动脉管壁有动脉粥样硬化改变的占88．3％。其中小动脉被完全阻塞的占10．0％(6侧)，部分阻塞占30．0％(18侧)。结论：50岁以上者出现视力下降、视野缺损，特别是双眼同向性偏盲，若排除其他疾患，则可能是脑底动脉粥样硬化引起外侧膝状体血供障碍所致。     

Langerin: a new lectin specific for Langerhans cells induces the formation of Birbeck granules

Generation of monoclonal antibodies restricted to human dendritic cells generated from CD34+ hematopoietic precursors has enabled the identification of Langerin, a Ca(++)-dependent type II lectin. Only expressed by Langerhans cells, Langerin is responsible for Birbeck granule formation by membrane superimposition and zippering. Furthermore, cell-surface Langerin is rapidly internalized into Birbeck granules, and does not colocalize with MHC class II rich compartments. Langerin gene transfected into mouse fibroblasts induces the formation of Birbeck granule-like structures, that would permit a better understanding of the function of Birbeck granules.     

Langerhans cell: CD1 antigens and the Birbeck granule

Langerhans cells are characterized by two types of markers: an ultrastructural marker, the Birbeck granule and different membrane markers: HLA-D antigens, T4 antigen, and some of the CD1 antigens. These antigens which are specific for the epidermal Langerhans cells, are not expressed by the other epidermal cells. Three CD1 antigens are biochemically defined on human thymocytes, they display a glycoprotein chain non covalently attached to beta-2-microglobulin. Only two of these glycoproteins: the T6 and M241 molecules have been detected on Langerhans cells. The presence of these CD1 antigens on Langerhans cells enhances their relationships to thymocytes, in contrast, Langerhans cells cannot be any more easily associated with the macrophage-monocyte lineage. The physiology of the ultrastructural marker is not well known. Its membrane origin has been experimentally proved since T6 antigen has been found closely associated to newly formed Birbeck granules.     

大鼠脾内含Birbeck颗粒的细胞

Birbeck颗粒是一种吸附、内包的细胞器,可能与抗原的提呈有关。它们最初被发现于表皮的郎格罕细胞中,以后又见于输入淋巴的面纱细胞以及胸腺和引流皮肤淋巴结的交错网状细胞。但至今尚未确认脾内有含Birbeck颗粒的细胞。我们采用Kamperdijk分离大鼠树状突细胞的方法(稍加改良)分离大鼠脾细胞,获得了富有树状突细胞的组分。电镜下,这些细胞的形态和结构与Steinman等描述的树状突细胞相似。少数细胞内含有Birbeck颗粒。颗粒呈棒状,外有一层界膜,内有一条电子致密的中柱,散布于细胞质内。本研究证明大鼠脾内有含Birbeck颗粒的细胞。     

HLA class II expression on human epidermal Langerhans cells in situ: upregulation during the elicitation of allergic contact dermatitis.

An immunoelectron-microscopic technique was applied to investigate the localization of molecules that are involved in the elicitation of allergic contact dermatitis in human epidermal cells in situ. Langerhans cells in the epidermis of lesions showed a strongly increased cell surface expression of HLA class II molecules as compared with normal skin. In addition, a high number of intracellularly located HLA class II molecules were present in Langerhans cells of lesional epidermis, suggesting increased biosynthesis of these molecules during the elicitation process. In contrast, no differences in the expression of CD1a by Langerhans cells was observed between normal and lesional skin. Frequently, the Langerhans cells were found in close apposition to mononuclear cells, which also exhibited a strong cell surface HLA class II expression. The number of Birbeck granules that are characteristic intracellular Langerhans cells organelles was increased in lesional Langerhans cells as compared with normal-skin Langerhans cells, which may correlate with the activated state of lesional Langerhans cells. These Birbeck granules were always HLA class II or CD1a negative. The increased synthesis and expression of HLA class II molecules on the cell surface of Langerhans cells suggests a direct role for these HLA class II molecules in the elicitation process of allergic contact dermatitis.     

食管上皮组织中的郎格罕氏细胞的电镜观察

运用透射电镜观察了8例正常食管上皮组织中的郎格罕氏细胞,较详细描述了其超微结构特点。结果再次提示郎格罕氏细胞胞浆内的Birbeck颗粒可能有两种形成途径。     

Coexpression of binding sites for A(B) histo-blood group trisaccharides with galectin-3 and Lag antigen in human Langerhans cells.

Galectin-3 is an immunomodulatory protein with binding capacity for various glycoconjugates including IgE. It has been shown to be produced by epidermal keratinocytes and is present on the surfaces of skin Langerhans cells (LC). Therefore, it may have a role in the pathogenesis of various skin diseases, such as atopic dermatitis. To study the expression of galectin-3 in LC, we used, in addition to specific antibodies, a panel of synthetic, carrier-immobilized, specific oligosaccharides of the A- and B-histo-blood group, which are recognized by this lectin. In the mean time, Birbeck granules were visualized with an anti-Lag antibody. The double labeling experiments showed a remarkable colocalization of signals for Lag antigen (Birbeck granules) and galectin-3, as well as the binding sites for A- and B-histo-blood group trisaccharides. The specificity of the oligosaccharide binding was demonstrated by the lack of binding by Le(c), Le(d) (H blood group antigen), and sLe(x), which are not recognized by galectin-3. These results suggest that galectin-3 is present in Birbeck granules, where it retains reactivity for its glycoligands.     

The Langerhans Cell: Sentinel of the Body's Immune Defense System. I. Morphology and Microscopic Methods of Detection

Abstract

The Langerhans cell (LC), although found in the skin, is one of the cells of the lymphoid system; it is believed to originate in the bone marrow. The LC wanders to other parts of the body, where it plays an important part in the immune response by helping to present antigen to T lymphocytes. Langerhans cells are differentiated from macrophages, melanocytes, T lymphocytes, and interdigitating cells by a number of markers, the most useful being OKT-6/Leu6 and the Birbeck granule. (The J Histotechnol 10: 121, 1987).     

Langerhans cells among bile duct epithelial cells in chronic liver disease

Langerhans cells were found among bile duct epithelial cells in a biopsy specimen from a patient with chronic liver disease showing cholangitic features. The bile duct was 90 micrometers in diameter and surrounded with mononuclear cell infiltration. Under the electron microscope, the cell had a clear cytoplasm and contained a deeply indented nucleus, a centriole, well-developed Golgi complexes and many rod-shaped bodies (Birbeck granules).     

LANGERHANS CELLS AMONG BILE DUCT EPITHELIAL CELLS IN CHRONIC LIVER DISEASE

Langerhans cells were found among bile duct epithelial cells in a biopsy specimen from a patient with chronic liver disease showing cholangitic features. The bile duct was 90 μm In diameter and surrounded with mononuclear cell infiltration. Under the electron microscope, the cell had a clear cytoplasm and contained a deeply indented nucleus, a centriole, well-developed Golgi complexes and many rod-shaped bodies (Birbeck granules).