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

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

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

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

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

OBJECTIVETo investigate the expressions of CD1a and CD83 of Langerhans cells (LC) in the lesions of epidermodysplasia verruciformis (EV) patients.

METHODSWe used immunohistochemical method to detect the expressions of CD1a and CD83 in the lesions of 10 patients with EV lesions and in the skins of 10 normal subjects.

RESULTSNo CD83 + LCs was detected in all EV patients and normal controls, but CD1a + LC was found in all cases. The quantity of CD1a + LCs in the lesions of EV patients was significantly lower than that in the normal skin (P < 0.01); furthermore, the distribution of LCs in EV lesions was uneven.

CONCLUSIONThe functions of LCs may be inhibited in EV patients.

Antigens, CD ; biosynthesis ; genetics ; Antigens, CD1 ; biosynthesis ; genetics ; Epidermodysplasia Verruciformis ; immunology ; pathology ; Humans ; Langerhans Cells ; immunology ; Leukocyte Immunoglobulin-like Receptor B1 ; Receptors, Immunologic ; biosynthesis ; genetics ; Skin ; immunology ; pathology

To study the effect of simian vacuolating virus 40 (SV40) on development and differentiation of dendritic cells (DC) from rhesus macaque, the peripheral blood-derived dendritic cells from rhesus monkey were pulsed with inactivated SV40 and infective SV40, respectively at the 5th day post DC cultivation. Expressions of CD1a, HLA-DR, CD86 and CD83 on the cell surface at the 7th, 9th day post DC cultivation were analyzed by flow cytometry (FCM). The results showed that expressions of CD1a, HLA-DR, CD86 and CD83 on the cell surface in the inactivated SV40-pulsed experimental group were higher than those in the infective SV40-pulsed experimental group (P < 0.05). These cell surface molecules represented characteristic development and differentiation phase of DC. Down-regulation of expressions of these cell surface molecules indicated that infective SV40 might hamper differentiation and maturation of dendritic cells from rhesus monkey.
Animals ; Antigens, CD ; metabolism ; Antigens, CD1 ; metabolism ; B7-2 Antigen ; metabolism ; Cell Differentiation ; Cells, Cultured ; Dendritic Cells ; cytology ; immunology ; virology ; Flow Cytometry ; HLA-DR Antigens ; metabolism ; Immunoglobulins ; metabolism ; Macaca mulatta ; Membrane Glycoproteins ; metabolism ; Polyomavirus Infections ; physiopathology ; Simian virus 40 ; physiology