Acupuncture Points ; Adult ; Bloodletting ; Conjunctiva ; immunology ; Conjunctivitis ; immunology ; therapy ; Humans ; Lymphatic Diseases ; immunology ; therapy ; Lymphatic Vessels ; immunology ; Male

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.
Autoimmune Diseases/*immunology ; Cholangitis, Sclerosing/*immunology ; Humans ; Immunoglobulin G/*immunology ; Lacrimal Apparatus/immunology ; Lymphatic Diseases/*immunology ; Pancreatitis, Chronic/*immunology ; Salivary Glands/immunology ; Sclerosis/immunology

OBJECTIVETo study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.

METHODSThe clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.

RESULTSThere were 9 male patients and 8 female patients. The age of patients ranged from 11 to 71 years (mean = 48.5 years). The main clinical manifestation was swelling of the eyelids: bilateral in 11 patients and unilateral in 6 patients. The duration of disease ranged from 5 months to 7 years (more than 2 years in 13 cases). Six patients had history of allergic disorders. In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney. The disease relapsed in 9 patients. Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission. The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L. Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case. There were various degrees of lymphoplasmacytic infiltrates (with lymphoid follicle formation) and stromal fibrosis. Classical obliterative phlebitis was absent. Tissue eosinophilia was demonstrated in the 17 cases studied. Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%.

CONCLUSIONThere are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease. Thorough understanding is important in arriving at a correct diagnosis.

Adult ; Aged ; Autoimmune Diseases ; immunology ; pathology ; Child ; Female ; Humans ; Immunoglobulin G ; Kidney Diseases ; immunology ; pathology ; Lung Diseases ; immunology ; pathology ; Lymphatic Diseases ; immunology ; pathology ; Male ; Middle Aged ; Orbital Diseases ; immunology ; pathology ; Plasma Cells ; immunology

Aged ; Castleman Disease ; immunology ; pathology ; Diagnosis, Differential ; Humans ; Immunoglobulin G ; metabolism ; Lymphatic Diseases ; immunology ; pathology ; surgery ; Lymphoma ; pathology ; Male ; Plasma Cells ; immunology ; Pseudolymphoma ; immunology ; pathology

Antigens, CD ; immunology ; Antigens, Differentiation, Myelomonocytic ; immunology ; Child, Preschool ; Diagnosis, Differential ; Histiocytes ; immunology ; pathology ; Histiocytosis, Sinus ; diagnosis ; pathology ; Humans ; Lymph Nodes ; immunology ; pathology ; Lymphatic Diseases ; diagnosis ; pathology ; Male

OBJECTIVETo observe the pathology of AIDS-related lymphadenopathy and its relationship to the expression and distribution of CD4 + CD25 + regulatory T cells in lymphoid node tissue.

METHODSTotally 22 biopsy and 13 autopsy lymphoid node tissues from HIV-positive patients were examined under microscopy and pathological staging was performed. Specific marker for CD4 + CD25 + regulatory T cells in lymphoid node tissue was detected with anti-Foxp3 monoclonal antibody by immunohistochemistry.

RESULTSAmong all the 35 specimens, 5, 4, 14, and 12 specimens were histopathologically staged from 1 to 4, respectively. FoxP3 were detected in all lymphoid node tissues. The distribution of FoxP3-positive lymphocytes were mainly in intermediate zone of follicle and cortical area in stages 1 and 2. The counts of FoxP3-positive lymphocytes remarkably decreased in stages 3 and 4, following depletion of lymphocytes.

CONCLUSIONSCD4 + CD25 + regulatory T cells exist in lymphoid node tissue of patients with HIV infection. Their amounts decrease or deplete along with the progression of AIDS-related lymphadenopathy.

Acquired Immunodeficiency Syndrome ; immunology ; pathology ; Adult ; CD4 Lymphocyte Count ; Female ; Forkhead Transcription Factors ; analysis ; Humans ; Immunohistochemistry ; Lymph Nodes ; immunology ; pathology ; Lymphatic Diseases ; immunology ; Male ; Middle Aged ; T-Lymphocytes, Regulatory ; metabolism

Fine-needle aspiration (FNA) of lymph nodes has been regarded as a useful method in the diagnosis of lymphadenopathy. However, this procedure has been shown to be of limited value in the diagnosis of low or intermediate grade malignant lymphomas in some studies. Immunophenotyping is an essential adjunct to cytomorphology for the diagnosis of lymphoma by FNA. Immunophenotyping using flow cytometry (FCM) is rapid, objective and reliable. Using FCM, multiparametric analysis of 33 FNA materials from lymph nodes was performed and profiles of surface markers of lymphoid cells were assessed. In reactive hyperplasia, patterns of cell surface markers were quite variable, but disclosed polyclonality. Most of the B-cell lymphomas showed immunophenotypes for B-cell lineages with their kappa: lambda or lambda: kappa ratio being over 3:1. In T-cell lymphomas, T-cell surface markers were predominantly expressed as well. In conclusion, our results suggest that immunophenotyping of lymph node aspirates is a valuable diagnostic adjunct for lymphoproliferative disorders, particularly in B-cell lymphomas because immunophenotyping can be easily and adequately performed by FCM.
Antigens, CD19/analysis ; Antigens, CD20/analysis ; Antigens, CD3/analysis ; Antigens, CD4/analysis ; Antigens, CD5/analysis ; Antigens, CD7/analysis ; Antigens, CD8/analysis ; B-Lymphocytes/immunology ; B-Lymphocytes/chemistry ; Biopsy, Needle ; Flow Cytometry/methods* ; Hodgkin Disease/pathology ; Human ; Immunophenotyping ; Lymph Nodes/pathology ; Lymph Nodes/chemistry ; Lymphatic Diseases/pathology* ; Lymphatic Metastasis/pathology ; Lymphoma, B-Cell/pathology* ; Lymphoma, Non-Hodgkin/pathology ; T-Lymphocytes/immunology ; T-Lymphocytes/chemistryt

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Castleman Disease ; immunology ; pathology ; Cyclophosphamide ; therapeutic use ; Diagnostic Errors ; Doxorubicin ; therapeutic use ; Humans ; Immunoglobulin G ; blood ; Kidney ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Lymphatic Diseases ; drug therapy ; immunology ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; Pancreas ; Plasma Cells ; immunology ; pathology ; Prednisone ; therapeutic use ; Submandibular Gland ; Vincristine ; therapeutic use