OBJECTIVETo study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).

METHODSThe expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSImmunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).

CONCLUSIONInflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; metabolism ; pathology ; Granulomatosis with Polyangiitis ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; Humans ; Immunoglobulin G ; metabolism ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; immunology ; pathology ; Nasal Polyps ; metabolism ; pathology ; Nose Diseases ; immunology ; pathology ; Paranasal Sinus Diseases ; immunology ; pathology ; Paranasal Sinuses ; immunology ; pathology ; Plasma Cells ; immunology ; Rhinoscleroma ; metabolism ; pathology ; Sinusitis ; metabolism ; pathology ; Young Adult

Aged, 80 and over ; Antibodies, Monoclonal ; metabolism ; Carcinoma, Squamous Cell ; metabolism ; pathology ; Diagnosis, Differential ; Granuloma ; metabolism ; pathology ; Humans ; Keratins ; immunology ; Male ; Mucin-1 ; metabolism ; Skin Neoplasms ; metabolism ; pathology

Adult ; Arthropathy, Neurogenic ; etiology ; immunology ; pathology ; Female ; Granuloma, Plasma Cell ; complications ; immunology ; pathology ; Humans ; Lung Neoplasms ; complications ; immunology ; pathology

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
Fatal Outcome ; Granuloma, Respiratory Tract/diagnosis/*etiology/therapy ; Humans ; Immunologic Deficiency Syndromes/*complications/immunology/pathology ; Lung Diseases/diagnosis/*etiology/therapy ; Male ; Middle Aged ; Thymoma/*complications/immunology/pathology ; Thymus Neoplasms/*complications/immunology/pathology

OBJECTIVETo study the clinicopathologic features of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen.

METHODSOne case of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen was examined macroscopically and microscopically. Immunohistochemical study for CD21, CD23, CD35, clusterin, S-100 protein, vimentin, smooth muscle actin, CD1a, CD68, ALK protein, CD30, CD31, CD34, CD3 and CD20 was performed on formalin-fixed, paraffin-embedded sections by standard EnVision method. In-situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was also carried out.

RESULTSMacroscopically, inflammatory pseudotumor-like follicular dendritic cell tumor was large in size, tan-colored, soft to rubbery in consistance and associated with central hemorrhage and necrosis. Histological examination showed scattered follicular dendritic cells admixed with abundant lymphocytes and plasma cells in the background, simulating inflammatory pseudotumor. On high-power magnification, the follicular dendritic cells possessed a moderate amount of pale to lightly eosinophilic cytoplasm, with indistinct cell borders. The nuclei were ovoid or spindly, with vesicular or stippled chromatin and small distinct, often centrally located, nucleoli. Some of the tumor cells showed nuclear pleomorphism and contained irregular foldings of nuclear membrane, coarse chromatin and prominent eosinophilic nucleoli. Mitotic figures were rarely identified. Immunohistochemical study showed that the tumor cells were positive for vimentin, clusterin, smooth muscle actin and CD68. They were weakly and focally positive for CD35 and S-100 protein, but negative for CD21, CD23, CD1a, ALK protein, CD30, CD31 and CD34. Most of the background lymphocytes were of T-lineage (CD3-positive) ,some were CD20 (B-cell marker)-positive. EBV RNA was demonstrated in the tumor cells by in-situ hybridization analysis.

CONCLUSIONSInflammatory pseudotumor-like follicular dendritic cell tumor is a rarely encountered low-grade malignancy with distinctive morphologic pattern. It is associated with EBV infection.

Adult ; Antigens, CD ; Antigens, Differentiation, Myelomonocytic ; Dendritic Cell Sarcoma, Follicular ; pathology ; physiopathology ; Dendritic Cells, Follicular ; pathology ; Female ; Granuloma, Plasma Cell ; etiology ; Herpesvirus 4, Human ; genetics ; immunology ; isolation & purification ; Humans ; Splenic Neoplasms ; pathology ; physiopathology

OBJECTIVETo observe the change in quantity and morphology of nerve fibers in different periods in granulation tissue in full-thickness burn wound.

METHODSThe granulation tissue samples were harvested from 40 patients with full-thickness burn in our unit at 1st, 2nd, 3rd and 4th post burn week (PBW), 10 samples were obtained at each time point. Donor site tissues from 10 burn patients were used as normal control. Immunofluorescent staining technique with anti-neurofilament (NF) monoclonal antibody was employed to examine the expression of nerve fibers in granulation tissue and normal skin. The morphology of nerve fibers was observed with fluorescence microscope and laser scanning confocal microscope.

RESULTSFluorescence microscopy showed: nerve fibers were short and rare at 1 PBW, the ratio of nerve fibers positive area was (0.14 +/- 0.08)%. Nerve fibers increased slightly and were in single filament without branches, and the positive area ratio of nerve fibers (0.40 +/- 0.09)% was much lower than that of normal control [(0.62 +/- 0.12)%, P < 0.05]. Nerve fibers increased significantly and were arranged like a mesh with more branches and sproutings, and the positive area ratio of nerve fibers was (0.73 +/- 0.16)% at 3 PBW. The quantity of nerve fibers at 4 PBW was similar to that of 3 PBW, and the positive area ratio of nerve fibers was (0.66 +/- 0.13)%. Observations under LSCM: the nerve fibers were short at 1, 2 PBW; was irregular at 3 PBW, among them some were swollen and distorted, and fragmentation and vacuolation were observed. They became aggregated at 4PBW with less branches, similar to that at 3 PBW. The structures of nerve fibers in normal control were intact, without obvious pathological changes.

CONCLUSIONThe change in quantity and morphology of nerve fibers in burn wound is related to the time of granulation tissue development.

Adult ; Burns ; pathology ; Female ; Fluorescent Antibody Technique ; Granuloma ; etiology ; pathology ; Humans ; Male ; Middle Aged ; Nerve Fibers ; metabolism ; pathology ; Nerve Regeneration ; Neurofilament Proteins ; immunology ; Skin ; innervation ; Wound Healing

BACKGROUND: Fibrin-ring granuloma (FRG), which can be found in bone marrow or the liver, is a subtype of epithelioid granuloma characterized by a central fat vacuole and annular peripheral fibrinoid materials. FRG has been proven to be associated with many etiologies such as several infectious organisms (Coxiella burnett; Epstein-Barr Virus, EBV; cytomegalovirus, CMV; and hepatitis A virus), allopurinol induced hepatitis, Hodgkin's lymphoma, and peripheral T-cell lymphoma. METHODS: We retrospectively reviewed 24 patients diagnosed with FRG by bone marrow biopsy at a single institute between 1995 and 2004. We reviewed clinical symptoms and laboratory findings of the patients, classified them by etiology, and compared prognosis of each group. RESULTS: The most common cause of FRG was acute or chronic EBV infection. Chronic or acute EBV infection was associated with 41.4% of patients (10/24). Of the remaining patients, 33.3% (8/24) were leukemia or lymphoma patients after chemotherapy, 4.2% (1/24) was a patient with hepatic failure, and 20.8% (5/24) were diagnosed as fever of unknown origin. The most common symptom and clinical finding were fever and cytopenia. EBV-associated group comprised chronic active EBV infection, EBV-associated hemophagocytic histiocytosis, acute EBV infection, EBV-associated lymphoproliferative disease, and Langerhans' cell histiocytosis. The EBV-associated group showed a lower survival probability compared with the non-EBV group (P<0.05). CONCLUSIONS: Patients with bone marrow fibrin ring granuloma accompanied by fever require an active workup to find out the cause of infectious agents including EBV infection particularly due to their poor prognosis.
Adolescent ; Adult ; Aged ; Bone Marrow Diseases/diagnosis/*etiology/pathology ; Child ; Child, Preschool ; Epstein-Barr Virus Infections/*complications/diagnosis ; Female ; Fibrin/analysis ; Granuloma/diagnosis/*etiology/pathology ; Herpesvirus 4, Human/immunology/isolation & purification ; Humans ; In Situ Hybridization ; Male ; Middle Aged ; Polymerase Chain Reaction ; Prognosis ; Q Fever/diagnosis ; Retrospective Studies ; Survival Rate

Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; administration & dosage ; Doxorubicin ; administration & dosage ; Female ; Granuloma ; drug therapy ; immunology ; pathology ; Humans ; Ki-1 Antigen ; analysis ; Lymphoma, Large-Cell, Anaplastic ; drug therapy ; immunology ; pathology ; Vincristine ; administration & dosage

OBJECTIVEInappropriate treatment at early stage of wound could result in the formation of pseudoepitheliomatous granuloma (PEG). The correlation of abnormal transdifferentiation of epithelial cells to immunologic cells and the occurrence of PEG lesion was investigated.

METHODSMorphological change of epithelial tissue was observed with histopathology in 11 specimens of PEG lesions and 6 specimens of normal skins from PEG edge (PEG-N) from 11 patients with damaged skin. The expression characteristics and distribution of pan-cytokeratin (CKp), IV type collagen, laminin (LM), epithelial cadherin (E-Cad), beta-catenin (beta-Cat), focal adhesion kinase (FAK), stem cell factor (SCF) and its receptor-c-Kit, proliferating cell nuclear antigen(PCNA), and cluster of differentiation-14 (CD14), CD68 and mast cell tryptase (MCT) in PEG were detected with the immunohistochemical and the indirect immunofluorescent double-staining.

RESULTSIn comparison with PEG-N, epithelial tissue take on squamous metaplasia, and stroma was infiltrated with intensive microvessels and inflammatory cells in the PEG lesion. Poor epithelial basal layer constitution, basal polarization, and migration of basal cells to stroma could be observed. In the ultrastructure, the loose intercellular junction of basal cells and the increased nucleus/cytoplasm ratio and intercellular space could be observed, neonatal monocytoid cells and macrophages and mast cells as a exuviate-like manner brooded from cytoplasm of original epithelial cells and basement membrane. protein expression of CKp and E-Cad by basal cells was significantly decreased, and the IV type collagen and LM protein could not be found in basement membrane of identical locus. By contrast, the immunoreactivity of beta-Cat and FAK was apparently increased. In addition, CD14(+) monocytes, CD68(+) macrophages, MCT(+) mast cells and CD68(+)/MCT(+) cells with various size, and these cells of stronger immuno-staining of SCF, c-Kit and PCNA antigen could be found in epithelial tissue and stroma.

CONCLUSIONEpithelial cells in PEG related to wound are characteristized by transdifferentiation of epithelial cells to immunologic cells, wich may be associated with local infectious and inflammatory reaction, ultimately resulting in enhancement the ratio of beta-Cat/E-Cad signal and activation SCF-c-Kit signal pathway. The phenomena of transdifferentiation epithelial cells in the PEG lesion will help to recognize of the neoplatic immune and trauma repair mechanism.

Adolescent ; Adult ; Aged ; Antigens, CD ; analysis ; Antigens, Differentiation, Myelomonocytic ; analysis ; Burns ; physiopathology ; Cadherins ; analysis ; Cell Differentiation ; immunology ; Child ; Child, Preschool ; Collagen Type IV ; analysis ; Cytoskeletal Proteins ; analysis ; Epithelial Cells ; chemistry ; metabolism ; Female ; Fluorescent Antibody Technique, Indirect ; Focal Adhesion Kinase 1 ; Focal Adhesion Protein-Tyrosine Kinases ; Granuloma ; pathology ; physiopathology ; Humans ; Immunohistochemistry ; Infant ; Keratins ; analysis ; Laminin ; analysis ; Lipopolysaccharide Receptors ; analysis ; Male ; Middle Aged ; Protein-Tyrosine Kinases ; analysis ; Proto-Oncogene Proteins c-kit ; analysis ; Serine Endopeptidases ; analysis ; Skin ; chemistry ; pathology ; physiopathology ; Stem Cell Factor ; analysis ; Trans-Activators ; analysis ; Tryptases ; beta Catenin

We report two cases of gingival plasma cell granuloma in a 34-yr-old and 40-yr-old two male renal transplant recipients with cyclosporine A (CsA)-induced gingival overgrowth (GO). Histologically, these lesions were composed of mature plasma cells, showing polyclonality for both lambda and kappa light chains and fibrovascular connective tissue stroma. In addition to the fact that CsA-induced plasma cell granuloma is rare, the salient features of our cases were the secretion of interleukin-6 and overexpression of phospholipase C-gamma1 of the tumor cells, which may explain the mechanisms of CsA- induced GO.
Cyclosporine/adverse effects ; Female ; Gingival Diseases/*chemically induced/enzymology/immunology/pathology ; Granuloma, Plasma Cell/*chemically induced/enzymology/immunology/pathology ; Humans ; Immunohistochemistry ; Immunosuppressive Agents/adverse effects ; Interleukin-6/metabolism ; Kidney Transplantation ; Male ; Middle Aged ; Phospholipase C gamma ; Type C Phospholipases/metabolism