OBJECTIVEThis review focuses on the current knowledge on the implication and significance of beta 2 microglobulin (β2M), a conservative immune molecule in vertebrate.

DATA SOURCESThe data used in this review were obtained from PubMed up to October 2015. Terms of β2M, immune response, and infection were used in the search.

STUDY SELECTIONSArticles related to β2M were retrieved and reviewed. Articles focusing on the characteristic and function of β2M were selected. The exclusion criteria of articles were that the studies on β2M-related molecules.

RESULTSβ2M is critical for the immune surveillance and modulation in vertebrate animals. The dysregulation of β2M is associated with multiple diseases, including endogenous and infectious diseases. β2M could directly participate in the development of cancer cells, and the level of β2M is deemed as a prognostic marker for several malignancies. It also involves in forming major histocompatibility complex (MHC class I or MHC I) or like heterodimers, covering from antigen presentation to immune homeostasis.

CONCLUSIONSBased on the characteristic of β2M, it or its signaling pathway has been targeted as biomedical or therapeutic tools. Moreover, β2M is highly conserved among different species, and overall structures are virtually identical, implying the versatility of β2M on applications.

Antigens, CD1 ; physiology ; Hemochromatosis Protein ; analysis ; Histocompatibility Antigens Class I ; physiology ; Humans ; Receptors, Fc ; physiology ; beta 2-Microglobulin ; blood ; chemistry ; deficiency ; physiology

OBJECTIVETo explore the clinicopathologic features, immunophenotype, differential diagnosis and gene mutation status of the Erdheim-Chester disease (ECD).

METHODSClinical and pathologic findings of 3 ECD cases were examined by gross, microscopic, immunohistochemical methods and BRAF V600E mutation. Related literatures were reviewed.

RESULTSTwo male patients and one female patient presented clinically with multiple skin nodules, bone pain and bony lesions by imaging study. Microscopically, the lesions were composed of spindle-shaped fibroblasts, foamy histiocytes and scattered Touton-type giant cells embedded in reactive fibrous tissue. Lymphocytes, plasma cells, and multinucleated giant cells were also found. Immunohistochemically, all histiocytes were positive for CD68, none of which expressed CD1a, although 2 cases focally expressed weak S-100 stain. In 2 cases,BRAF V600E mutation was detected.

CONCLUSIONSECD is a rare disease of xanthogranulomatous histiocytosis.Its diagnosis relies on pathological and immunohistochemical findings, but correlation with clinical information, especially radiographic findings should be performed.No effective treatment of the disease is currently available.

Antigens, CD ; analysis ; Antigens, CD1 ; analysis ; Antigens, Differentiation, Myelomonocytic ; analysis ; Diagnosis, Differential ; Erdheim-Chester Disease ; genetics ; immunology ; pathology ; Female ; Humans ; Male ; Mutation ; S100 Proteins ; analysis ; Treatment Outcome

A 56-year-old male underwent a screening colonoscopy. An 8-mm sessile polyp was removed from the descending colon using snare polypectomy. Histology showed Langerhans cells and eosinophil infiltration of the submucosa. Immunohistochemical staining was positive for S-100 protein and CD1a antigen, which confirmed the diagnosis of Langerhans-cell histiocytosis. On further workup, there was no evidence of involvement of any other organs. Here, we report a very rare case of colonic Langerhans-cell histiocytosis presenting as an isolated polyp.
Antigens, CD1 ; Colon ; Colon, Descending ; Colonoscopy ; Eosinophils ; Histiocytosis, Langerhans-Cell ; Humans ; Langerhans Cells ; Male ; Mass Screening ; Polyps ; S100 Proteins ; SNARE Proteins

Calreticulin (CRT) is a multifunctional molecule in both intracellular and extracellular environment. We have previously found that a recombinant CRT fragment (rCRT/39-272) could modulate T cell-mediated immunity in mice via activation and expansion of CD1d(hi)CD5⁺ B cells as well as induction of CRT-specific regulatory antibodies. Antibody secreting cells (ASCs) are terminally differentiated B cells responsible for producing antibodies to participate in positive immune response as well as immune regulation. In this study, we demonstrate that rCRT/39-272 differentiates murine CD1d(hi)CD5⁺ B cells into ASCs marked by increased expression of plasma cell-associated transcription factors and production of polyreactive antibodies against DNA and CRT in vitro. Intraperitoneal administration of rCRT/39-272 augmented differentiation of CD1d(hi)CD5⁺ B cells into ASCs in naïve mice or mice with experimental autoimmune encephalomyelitis. Thus, we propose that ASC differentiation and subsequent antibody production of CD1d(hi)CD5⁺ B cells are key steps in CRT-mediated immunoregulation on inflammatory T cell responses.
Animals ; Antigens, CD1d ; metabolism ; Autoantibodies ; biosynthesis ; B-Lymphocytes ; cytology ; drug effects ; immunology ; metabolism ; CD5 Antigens ; metabolism ; Calreticulin ; chemistry ; Cell Differentiation ; drug effects ; Encephalomyelitis, Autoimmune, Experimental ; immunology ; Humans ; Mice ; Peptide Fragments ; chemistry ; pharmacology ; Solubility

OBJECTIVESTo observe the clinicopathologic features of Langerhans cell histiocytosis (LCH), and to evaluate the values of langerin, CD1a and S-100 protein expression in diagnosis of the tumor.

METHODSTotal 258 cases of Langerhans cell histiocytosis in the past 18 years (from 1992 to 2008) were collected, morphologic review and immunohistochemical staining were performed.

RESULTSIn all 258 cases, the ages of patients older than 16 years or younger than 2 years were 126 (48.8%) and 37 (14.3%), respectively, in the remaining 95 (36.8%) of the cases, the age of the patients ranged from 2 to 16 years. For all of 258 cases, there were 364 diseased sites. Bony lesions accounted for 77.2% (281 cases), especially the skull (112 cases, 39.9%), followed by lymph node (25 cases, 6.9%) and skin (14 cases, 3.8%). Clinically, unisystem or unifocal disease was predominant (201 cases, 77.9%), followed by unisystem and multifocal disease (21 cases, 8.1%), multi-system disease (26 cases, 10.1%), isolated pulmonary LCH (2 cases, 0.8%), and unclassified (8 cases, 3.1%). Histologically, variable number of Langerhans cells was present in 265 samples of 258 cases. Multinucleated giant cells were found in 166 (62.6%) of the samples. Eosinophils were the major infiltrating non-neoplastic cells, and eosinophilic abscess was seen in 57 cases (21.5%). Coagulative necrosis and dead bone were detected in 29 (10.9%) and 124 (46.8%) of the cases, respectively. Immunohistochemically, the expression of S-100 protein, CD1a and langerin was 99.1% (209/211), 100% (206/206) and 98.5% (193/196), respectively, and the sensitivity of them had no statistical difference.

CONCLUSIONSIn this group of LCH cases, the ratio of adult patients is high, but the proportion of multi-organ lesion is low. No significant difference of the sensitivity is found among langerin, CD1a and S-100 expression in diagnosis of LCH.

Adolescent ; Adult ; Antigens, CD ; metabolism ; Antigens, CD1 ; metabolism ; Child ; Child, Preschool ; Diagnosis, Differential ; Eosinophils ; pathology ; Female ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Infant ; Langerhans Cells ; pathology ; Lectins, C-Type ; metabolism ; Lymph Nodes ; pathology ; Male ; Mannose-Binding Lectins ; metabolism ; Middle Aged ; S100 Proteins ; metabolism ; Skin ; pathology ; Survival Rate ; Young Adult

We report a case of Langerhans cell sarcoma presented as a solitary mass in the left supraclavicular area in a 31-year-old woman. Computed tomography revealed a relatively well-defined and lightly enhancing mass in the left supraclavicular area, measuring 5.5x4.5x3.2 cm. Excision was subsequently performed. Microscopically, the specimen consisted of an enlarged and partially effaced lymph node. Nests of different size composed of atypical tumor cells were located in the paracortex and the medulla of the lymph node. The tumor cells exhibited abundant eosinophilic or clear cytoplasm and displayed marked nuclear atypia and increased mitotic figures. Infiltration of many eosinophils was identified in the periphery and between the tumor cells. The tumor cells were reactive for CD1a and S100 protein. Ultrastructually, they were found to have Birbeck granules in the cytoplasm.
Adult ; Antigens, CD1 ; Cytoplasm ; Eosinophils ; Female ; Humans ; Langerhans Cell Sarcoma ; Lymph Nodes

Adolescent ; Adult ; Antigens, CD ; metabolism ; Antigens, CD1 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Histiocytosis, Langerhans-Cell ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Infant ; Lectins, C-Type ; metabolism ; Male ; Mannose-Binding Lectins ; metabolism ; Middle Aged ; S100 Proteins ; metabolism ; Survival Rate ; Young Adult

OBJECTIVETo study clinicopathological features, diagnosis, differential diagnosis of oral Langerhans cell histiocytosis (LCH), retrospective clinicopathologic study was carried on and a variety of immune phenotype were detected.

METHODSThe clinicopathological features of 29 cases of oral LCH were analyzed. The immunohistochemical staining of S-100 protein, CD1a, CD83 and Ki-67 were used in above cases by immunohistochemical streptavidin-biotin peroxidase (SP) and Elivison two-step method. Statistical analysis was adopted for the results.

RESULTSOf the 29 cases of LCH, the expression of S-100 protein and CD1a were positive in 24 cases and negative in 5 cases, so 5 cases were excluded from the diagnosis of LCH. Among 24 cases of LCH, 15 patients were male and 9 were female. The median age was 7.50 years. 14 lesions were in the mandible, 5 were in the maxilla and 5 involved the mandible and maxilla. 9 cases were in stage I, 13 in stage II and 2 in stage III, according to Bartnick classification. Immunohistochemistry showed all 24 cases staining for S-100 protein and CD1a were positive. Comparing with maxillofacial lesions involved soft tissue, Ki-67 positive rate was lower and CD83 positive rate was higher in maxillofacial single bone lesion.

CONCLUSIONThe immunohistochemical staining of S-100 protein and CD1a are important for the diagnosis of LCH. Maxillofacial bone single LCH might have lower proliferative activity and a higher state of maturity. Maxillofacial LCH involved soft tissue might have a higher proliferative activity and a lower state of maturity.

Antigens, CD1 ; Diagnosis, Differential ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Immunohistochemistry ; Male ; Mandible ; Maxilla ; Retrospective Studies ; S100 Proteins

TGF-beta-induced tolerogenic-antigen presenting cells (Tol-APCs) could induce suppression of autoimmune diseases such as collagen-induced arthritis (CIA) and allergic asthma. In contrast, many studies have shown that NKT cells are involved in the pathogenesis of Th1-mediated autoimmune joint inflammation and Th2-mediated allergic pulmonary inflammation. In this study, we investigated the effect of CD1d-restricted NKT cells in the Tol-APCs-mediated suppression of autoimmune disease using a murine CIA model. When CIA-induced mice were treated with Tol-APCs obtained from CD1d+/- or CD1d-/- mice, unlike CD1d+/- APCs, CD1d-/- Tol-APCs failed to suppress CIA. More specifically, CD1d-/- Tol-APCs failed to suppress the production of inflammatory cytokines and the induction of Th2 responses by antigen-specific CD4 T cells both in vitro and in vivo. Our results demonstrate that the presence of CD1d-restricted NKT cells is critical for the induction of Tol-APCs-mediated suppression of CIA.
Animals ; Antibodies/blood ; Antibody Formation/immunology ; Antibody Specificity/immunology ; Antigen-Presenting Cells/*immunology ; Antigens, CD1d/immunology ; Arthritis, Experimental/blood/*immunology/*prevention & control ; Collagen Type II/immunology ; Cytokines/blood ; Immune Tolerance/*immunology ; Inflammation Mediators/blood ; Mice ; Natural Killer T-Cells/*immunology ; Th1 Cells/immunology

BACKGROUNDChronic obstructive pulmonary disease (COPD) is thought to be an inflammatory immune response disease. In most cases, the disease is caused by cigarette smoke, but it has been demonstrated that only 10% to 20% of smokers will definitely suffer from COPD. Dendritic cells (DCs) are considered to be the promoter of immune responses. However, the underlying mechanisms involved are still unrevealed. In this study, we aimed to investigate the quantitative differentiation of pulmonary DC in smokers with or without COPD to explore the possible role of DCs in smokers suffering COPD.

METHODSPeripheral lung specimens from non-smokers without airflow obstruction (control group, n = 7), smokers without airflow obstruction (smoker group, n = 7) and patients with COPD (COPD group, n = 7) were investigated to detect the quantity of S-100 and CD1a positive cells by immunohistochemical or immunofluorescent assay.

RESULTSIn smokers with COPD, the number of S-100(+) DCs was higher than in the controls and smokers without COPD (P < 0.01 and P < 0.05) and there was a higher number of S-100(+) DCs in smokers with COPD than in smokers without COPD, but without a significant difference (P > 0.05). An inverse correlation was found between the number of DCs and forced expiratory volume in the first second (FEV(1))% pred (r = -0.75, P < 0.05), which was also found between the number of DCs and FEV(1)/forced vital capacity (FVC) (r = -0.72, P < 0.05). The mean number of CD1a(+) DCs, increased from non-smokers to non-COPD smokers to COPD patients, with significant differences between each group (P < 0.01).

CONCLUSIONSThe quantity of DCs significantly increased in smokers with COPD compared with non-smokers or smokers without COPD. The results suggest that DCs may play an important role in the pathogenesis of smoking-induced COPD, and the upregulation of DCs may be a potential maker to identify the smokers who have more liability to suffer from COPD.

Aged ; Antigens, CD1 ; metabolism ; Cell Differentiation ; physiology ; Dendritic Cells ; cytology ; Female ; Fluorescent Antibody Technique ; Humans ; Immunohistochemistry ; In Vitro Techniques ; Lung ; metabolism ; pathology ; Male ; Microscopy, Confocal ; Middle Aged ; Pulmonary Disease, Chronic Obstructive ; metabolism ; pathology ; S100 Proteins ; metabolism ; Smoking ; adverse effects