Langerin在Langerhans组织细胞增生症和肺炎性与感染性疾病中的免疫组化分析

肺Langerhans组织细胞增生症（PLCH）是一种特发性间质性肺疾病，主要发生于30—40岁成人吸烟者。组织学上，PLCH表现为Langerhans细胞围绕远端呼吸道呈结节性间质性增生伴不同程度的嗜酸性粒细胞、淋巴细胞和巨噬细胞浸润，可见间质纤维化、水肿和支气管炎等病理改变。然而这些病理变化是非特异性的，可与其他的弥漫性间质性肺疾病如脱屑性间质性肺炎／呼吸性支气管炎相关性间质性肺疾病（DIP／RB．ILD）、普通性间质性肺炎、[第一段]     

胃黏膜Langerhans细胞组织细胞增生症

患者女性，59岁。因胃部不适、消化不良3个月余，于2002年8月9日来我院就诊。胃镜检查发现胃底、胃体中部散在9个大小不等的溃疡性病灶。小者直径约0．5cm，局部隆起，表面充血、浅表溃疡形成；大者位于贲门口和胃体大弯侧，直径达4cm，周边隆起，溃疡中心附着黄白脓苔。在贲门、胃体大溃疡和胃底小溃疡边缘分别行多块组织活检。     

淋巴结Langerhans细胞组织细胞增生症临床病理及？…

研究以淋巴结首发的ＬＣＨ形态结构特征,免疫表型,临床表现及鉴别诊断。方法应用ＨＥ染色和免疫组化ＡＢＣ法标记Ｏ１０和Ｓ－１００阳性以确定诊断。结果：１４例淋巴结ＬＣＨ有４种组合类型（１）肾形及卵圆形细胞４例;（２）咖啡豆样细胞３例;（３）咖啡豆样及大圆形细胞５例;（４）大圆形细胞２例。     

婴儿上纵隔Langerhans细胞组织细胞增生症及讨论

Langerhans细胞组织细胞增生症是以Langerhans细胞(LC)异常增生为特点的一组比较少见的、原因不明的疾病.年龄不同,患者的病变范围不同,临床表现也多种多样.本文报道发生于2个月婴儿上纵隔单发的Langerhans细胞组织细胞增生症1例,并复习有关文献.     

婴儿上纵隔Langerhans细胞组织细胞增生症及讨论

Langerhans细胞组织细胞增生症是以Langerhans细胞 (LC)异常增生为特点的一组比较少见的、原因不明的疾病 .年龄不同 ,患者的病变范围不同 ,临床表现也多种多样 .本文报道发生于 2个月婴儿上纵隔单发的Langerhans细胞组织细胞增生症 1例 ,并复习有关文献 .     

原发于胸腺的Langerhans细胞组织细胞增生症合并重症肌无力一例

患者女,28岁,因眼睑下垂伴复视3个月入院,查体：右眼睑下垂。CT扫描示右胸腺增生,入院诊断为重症肌无力（眼型）。于2004年5月9日行纵隔肿块切除术,术中见胸腺明显增生,与右侧胸膜稍粘连。     

树突状细胞摄取和提呈颗粒化抗原的能力与颗粒载体的大小相关

目的：研究体外小鼠骨髓树突状细胞对2种不同大小bead-OVA复合物（0.04 μm bead和1.0μm bead）的摄取及class I途径抗原提呈能力。方法：以2h骨髓粘附细胞为前体细胞，用GM－CSF（1000U／ml）和IL－3（10ng/ml）培养5d，观察细胞对FITC标记的2种bead-OVA复合物的摄取，PMA、amiloride、cytochalasin D对摄取的抑制，以及细胞摄取后表达MHC分子和共刺激分子的情况，同时用OVA表位特异性T细胞杂交检测细胞摄取后通过class I途径活化CTL应答的能力。结果：树突状细胞对1．0μm bead-OVA的摄取明显高于对0．04μm bead-OVA，前者被上述3种抑制剂显著抑制，后者仅对amiloride和PMA抑制作用敏感，CCD无明显抑制作用。与摄取结果相反，0．04μm bead－OVA较1．0μm bead－OVA诱导更强的CD8细胞免疫应答，表型分析显示，细胞摄取0．04μm bead后，MHC分子和共刺激分子表达显著高于1．0μm的bead。结论：树突状细胞对2种bead的摄取能力和摄取机制不一样，0．04μm bead尽管摄取效率不如1．0μm bead，但通过class I途径提呈抗原的效率显著高于后者。     

082 树突状细胞与髓性白血病的免疫治疗研究进展

树突状细胞（ＣＤ）是目前发现的功能最强的抗原提呈细胞（ＡＰＣ）,近年来对ＤＣ的研究成为热点。髓性白血病是一组不同分化程度多克隆的异常髓系增生性疾病。本文拟对ＤＣ应用于髓性白血病免疫治疗方面的研究进展作一综述。     

树突状细胞对外源性抗原的摄取和加工机制

树突状细胞 (dendriticcell,DC)是体内功能最强大的专职性抗原提呈细胞 (antigenpresentingcell,APC) ,因其成熟时胞体伸出许多树突状或伪足样突起而得名 ,与其它APC相比 ,DC能高效地内吞、处理及呈递抗原 ,并能激活幼稚型 (na¨lve)T淋巴细胞 ,启动初级免疫应答 ,故在免疫反应中占有极其特殊的地位[1] 。因此 ,深入了解DC对抗原的摄取、加工和MHC分子的生成及作用对揭示DC的抗原提呈机制有重要意义     

树突状细胞在调节性T细胞产生中的作用

树突状细胞（Dendritic cells，DCs）是一类重要的专职抗原提呈细胞，虽在体内的数量较少，但具有超强的抗原提呈能力，而且能够活化初始T细胞（Naive T cells），在免疫应答的诱导中具有独特的地位。DCs既能诱导有效的免疫应答，同时在诱导免疫耐受中也十分重要。在中枢耐受中，胸腺内的DCs可以通过阴性选择清除自身反应性T细胞。近年来，越来越多的证据表明，DCs在外周耐受中也起着关键性的作用，可以通过诱导效应性CD4^＋T或者CD8^＋T细胞的无能，促进调节性T细胞（T regulatory cells，Treg）的分化来调控针对自身抗原的外周耐受。DCs的来源、表型和成熟状态都具有多样性和异质性，DCs的耐受功能可能依赖于其某一成熟阶段或者是某一亚群。本文就DCs在诱导产生Treg中的作用和应用进展做一简要综述。     

Immunohistochemical study on antigenic phenotype of Langerhans cell histiocytosis.

Immunohistochemical study on 26 cases of Langerhans cell histiocytosis (LCH) using several leukocyte antibodies in addition to traditionally used markers (S-100 protein and peanut agglutinin) revealed that the proliferating cells of LCH expressed UCHL1, MT1 as well as classically known positivity for S-100 protein, HLA-DR and peanut agglutinin but were negative for OPD4. In comparison to S-100 protein peanut agglutinin (PNA) using a two stage method produced weaker staining and positively stained cells were sparse. Also in this study, a small proportion of proliferating cells in LCH was observed to be reactive for both myeloid/macrophage antigens (KPI, MAC 387 and lysozyme) and Langerhans cell marker (S-100 protein), verifying the existence of a hybrid form of histiocytes.     

Immunohistochemical characterization of immune cell infiltration in paediatric and adult Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia commonly affecting children with frequent somatic mutations in MAPK pathway genes including BRAF^V600E and MAP2K1. Some studies suggest that LCH cells can recruit and modulate inflammatory cells, which could provide reciprocal survival signals. To characterize the immune profile of infiltrating inflammatory cells, and to clarify their participation in LCH pathogenesis, a detailed immunohistochemical analysis was performed. Fifteen (10 children, 5 adults) LCH cases were assessed through macrophage (CD68 and CD163), mature dendritic cell (mDC; CD83 and CD208), regulatory T cell (Treg; CD4, CD25 and FOXP3) and cytotoxic lymphocyte (CL; CD56, CD57, perforin and granzyme B) immunomarkers. Moreover, lymphocytic and LCH markers were also analysed. All cases were S100, CD1a, CD207 and CD4-positive. Bcl-2 and cyclin D1 expression was observed in 13 of 15 cases. In the immune microenvironment, M2-polarized macrophages and Tregs were the predominant cell populations, followed by significantly (P < .005) smaller levels of mDCs and CLs. Additionally, the number of CD3 + cells was significantly higher than that of CD20 + cells. In the CD3 + cell population, there were a significantly higher number of CD4 + cells than CD8 + cells. While there were no differences when comparing the paediatric and adult populations, FOXP3 + cells were significantly higher in patients with multisystem involvement and treated with chemotherapy, than single-site cases and those without chemotherapy. Our results suggest that M2-polarized macrophages and Treg infiltration can promote LCH development and survival, probably through pro-tumoral, immunosuppressive and/or cytokine-mediated mechanisms. This work highlights the need for further exploration of immune-targeted therapy for LCH.     

“全国乳腺及生殖系统病理诊断与细胞学技术研讨会”将在深圳举办

目前，病理医师在外检工作中实际应用最新版《WHO肿瘤病理学及遗传学新分类》——乳腺和女性生殖系统病理分类标准时还存在一定难度，常造成病理诊断结果的差异，因此，深入探讨和系统理解WHO乳腺和女性生殖系统病变病理诊断的新进展、新标准具有重要现实意义。另外，近年来液基薄层细胞学检查技术方兴未艾，但实验方法及诊断标准较为混乱，也有待于进一步规范和统一。     

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disorder affecting predominantly children and manifesting as bone pains, bony swellings and lytic lesions. Involvement of vertebrae as presenting manifestation is unusual. Here we have presented three cases of LCH, two of multifocal eosinophilic granuloma (MEG) and one of Hand Schuller Christian disease (HSC). One of the patients with MEG; had vertebral involvement as the presenting manifestation.     

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare clonal disorder that consists of single or multiple mass lesions composed of cells with an abnormal Langerhans cell phenotype. Its etiology remains unknown, despite extensive searches for evidence of consistent cytogenetic abnormalities, gene rearrangements, or viral genomes. Similarly, the pathogenesis of the disease is enigmatic, although the altered expression of cytokines and cellular adhesion molecules, important for migration and homing of the activated normal Langerhans cell, may play an important role. The biologic behavior of LCH ranges from spontaneous remission to lethal dissemination, and such behavior cannot be predicted on the basis of histologic features. The presence and degree of organ dysfunction, together with the patient's age at diagnosis, remain the most reliable indicators of prognosis. Treatment of severe, refractory disease continues to be controversial and, in many cases, ineffectual. The revised classification scheme for LCH and related disorders recognizes the uncertain biological potential of LCH and its relation to other processes of dendritic and macrophage origin.     

Gene expression analysis of dendritic/Langerhans cells and Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a neoplastic disorder that results in clonal proliferation of cells with a Langerhans cell (LC) phenotype. The pathogenesis of LCH is still poorly understood. In the present study, serial analysis of gene expression (SAGE) was applied to LCs generated from umbilical cord blood CD34+ progenitor cells to identify LC-specific genes and the expression of these genes in LCH was investigated. Besides the expression of several genes known to be highly expressed in LCs and LCH such as CD1a, LYZ, and CD207, high expression of genes not previously reported to be expressed in LCs, such as GSN, MMP12, CCL17, and CCL22, was also identified. Further analysis of these genes by quantitative RT-PCR revealed high expression of FSCN1 and GSN in all 12 LCH cases analysed; of CD207, MMP12, CCL22, and CD1a in the majority of these cases; and CCL17 in three of the 12 cases. Immunohistochemistry confirmed protein expression in the majority of cases. The expression of MMP12 was most abundant in multi-system LCH, which is the LCH type with the worst prognosis. This suggests that expression of MMP12 may play a role in the progression of LCH. These data reveal new insight into the pathology of LCH and provide new starting points for further investigation of this clonal proliferative disorder.     

CD101 expression by Langerhans cell histiocytosis cells

AIMS: Our objective was to study the expression of a recently identified cell surface molecule, CD101 and in Langerhans cell histiocytosis (LCH) patients as CD101 has been shown to be present on dendritic cells. We wanted to determine if CD101 expression could be helpful for the diagnosis of LCH in conjunction with other markers (CD1a, S100 protein), and could be predictive of the evolution and dissemination of the disease. METHODS AND RESULTS: The expression of CD101 was studied by immunohistochemical technique in 11 cases of Langerhans cell histiocytosis on frozen sections. The expression of CD101 was positive in nine cases, high in six cases and low in three cases. There was no expression in the other two cases. No correlation with the evolution, the localization or the dissemination of the disease could be evidenced. CONCLUSIONS: CD101 is a new phenotypic marker that might be useful in combination with other markers for the diagnosis of LCH. However, as the anti-CD101 antibody works only in frozen sections, its value is limited compared to anti-CD1a antibody.