Abdominal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Adult ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Ki-1 Antigen ; metabolism ; Leukocytosis ; complications ; metabolism ; pathology ; surgery ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Young Adult

OBJECTIVETo study the clinical pathological features and immunophenotype of follicular dendritic cell sarcoma (FDCS) with discussion on its diagnostic clues to improve diagnostic level.

METHODSFive cases of FDCS were analyzed by clinical, pathologic and immunohistochemistry methods.

RESULTSFive cases of FDCS were located in the cervical lymph node. Microscopically, the normal architectures were effaced by ovoid, spindle-shaped with fascicular, diffuse or whorled patterns and with rich lightly eosinophilic cytoplasm, syncytial appearance. Nuclei tend to show irregular clustering, scattered multinucleated giant cell. Nucleoli often distinct, sometimes prominent. Mitotic count variable, may show significant cellular pleomorphism. Immunohistochemical studies show that the tumor cells were positive for CD21, CD35, but negative for CD1a, CD34, CK and HMB45. Under electron microscopy, the tumor cells possessed long villus cytoplasmic processes and desmosome-like junctions, Birbeck granules were absent.

CONCLUSIONSFDCS is a rare malignant tumor and differential diagnosis includes Langerhans cell sarcoma, interdigitating dentric cell sarcoma, malignant fibrous histocytoma, melanoma, metastatic spindle cell carcinoma and others. Immunohistochemistry and electron microscopy are necessary for a correct diagnosis.

Adult ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lymph Nodes ; metabolism ; pathology ; ultrastructure ; Male ; Microscopy, Electron ; Middle Aged ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism

BACKGROUNDComplement receptor type 2 (CR2) is the receptor for C3d and C3dg and for Epstein-Barr virus. The aim of our study was to explore whether CR2 can independently mediate the activation of mitogen-activated protein kinases (MAPKs, including ERK, JNK, and p38MAPK), and to highlight the molecular mechanism of CD4+ cell deletion in AIDS.

METHODSHOS cells (HOS-CR2) and HOS-CD4 cells (HOS-CD4CR2) stably expressing CR2 were established and then identified by FACS and Western blotting. Activation and blocking tests of MAPKs were assessed by Western blot. Cell proliferation was determined using Cell Titer 96((R)) Aqueous One Solution Reagent.

RESULTSFACS results showed that the positive rates of HOS-CR2 and HOS-CD4CR2 cells were greater than 96%, and Western blot showed that the CR2 expression levels on HOS-CR2 and HOS-CD4CR2 cells were high. Activation and blocking tests of MAPKs (ERK, JNK, and p38MAPK) were carried out in HOS-CR2, HOS-CD4, and HOS-CD4CR2 cells. The activation of MAPKs in HOS-CR2 cells stimulated with PMA (100 ng/ml) and NHS (10%) was identical. The activation of MAPKs increased at 5 minutes, reached a peak at 10 minutes, and decreased to baseline within 30 minutes, all in a time-dependent manner; the activation of MAPKs was blocked by anti-CR2 McAb, PD98059 (inhibitor of ERK), and Wortmanin (inhibitor of PI-3K), respectively. In HOS-CD4 cells, MAPKs were activated by HIV-gp160. In HOS-CD4CR2 cells, MAPK activation was induced by HIV-gp160, 10% NHS, and HIV-gp160 + 10% NHS; phosphorylation of p38MAPK was dramatically induced by HIV-gp160 + NHS, and lasted for 1 hour. The cell proliferation results showed that HIV-gp160 inhibited the proliferation of HOS-CD4 and HOS-CD4CR2 cells (P < 0.01) and that NHS enhanced the effect of HIV-gp160 (P < 0.01).

CONCLUSIONSThe activation of MAPKs is independently mediated by CR2 and that anti-CR2 McAb, PD98059, and Wortmanin block the activation of MAPKs, respectively. The results of the signal transduction and cell proliferation assays of HOS-CD4CR2 cells show that CR2 plays a role in the pathogenesis of HIV infection, especially in the inhibition of CD4+ cell proliferation.

Cell Division ; Cells, Cultured ; Enzyme Activation ; Flavonoids ; pharmacology ; HIV Envelope Protein gp160 ; pharmacology ; Humans ; Mitogen-Activated Protein Kinases ; metabolism ; Receptors, Complement 3d ; physiology ; Signal Transduction

OBJECTIVETo study the morphologic and immunophenotypic features of angioimmunoblastic T-cell lymphoma (AITL), as well as the origin of the proliferative follicular dendritic cells (FDCs) in AITL.

METHODSImmunohistochemical study for CD10, CXCL13, bcl-6 and CD21 was performed on 29 cases of AITL. Double immunostaining for bcl-6/CD3, CD10/CD21 and CD10/CD20 were also carried out. Cases of peripheral T-cell lymphoma, unspecified, extranodal NK/T-cell lymphoma, nasal-type, enteropathy-type T-cell lymphoma, anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma and reactive lymphoid proliferation were selected as controls.

RESULTSAmongst the 29 cases of AITL studied, 75.9% (22/29) showed aberrant expression of CD10, while all except one of the controlled cases were negative, 82.8% (24/29) of the AITL cases expressed CXCL13, while all cases of peripheral T-cell lymphoma, unspecified were negative. As for bcl-6 staining, although the highest percentage of bcl-6-positive cells was observed in AITL, the expression pattern was not useful in differentiating AITL from peripheral T-cell lymphoma, unspecified and lymphoid reaction. Besides, all cases of AITL demonstrated the characteristic proliferation of follicular dendritic cells. Two of the cases, which contained obvious germinal centers, had the follicular dendritic cell meshwork extending beyond the lymphoid follicles.

CONCLUSIONSAs compared with bcl-6, CD10 and CXCL13 are specific and sensitive markers in diagnosing AITL. Part of the proliferative FDCs in AITL may originate from the germinal centers.

Adult ; Aged ; Chemokine CXCL13 ; metabolism ; Dendritic Cells, Follicular ; metabolism ; pathology ; ultrastructure ; Female ; Humans ; Immunoblastic Lymphadenopathy ; metabolism ; pathology ; Immunophenotyping ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; Neprilysin ; metabolism ; Proto-Oncogene Proteins c-bcl-6 ; metabolism ; Receptors, Complement 3d ; metabolism

Back ; Follow-Up Studies ; Humans ; Lymphoma, Follicular ; metabolism ; pathology ; Male ; Middle Aged ; Neprilysin ; metabolism ; Proto-Oncogene Proteins c-bcl-6 ; metabolism ; Receptors, Complement 3d ; metabolism ; Skin Neoplasms ; metabolism ; pathology

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).

CONCLUSIONSFDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult

Abdominal Neoplasms ; drug therapy ; metabolism ; pathology ; secondary ; surgery ; Dendritic Cell Sarcoma, Follicular ; drug therapy ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Middle Aged ; Neoplasm Recurrence, Local ; Omentum ; Peritoneal Neoplasms ; secondary ; Receptor, Epidermal Growth Factor ; metabolism ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism

We investigated the clinical and biological significance of germinal centers (GC) present in the minor salivary glands of patients with Sjogren's syndrome (SS). Minor salivary gland tissue biopsies from 93 patients with SS were used to identify GC-like structures, which were confirmed by CD21-positive follicular dendritic cell networks. Patients were compared based upon sociodemographics, glandular and extraglandular manifestations, and laboratory findings including autoantibody profiles, complement, and immunoglobulin levels; EULAR SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. GC-like structures were observed in 28 of 93 SS patients (30.1%). Mean focus scores and CRP levels were significantly higher in GC-positive patients than in GC-negative patients; GC-positive patients also exhibit a higher prevalence of rheumatoid factor and anti-SS-A/Ro antibodies compared to GC-negative patients. No differences in glandular or extra-glandular manifestations were evident between groups. In conclusion, SS patients with GC-like structures in the minor salivary glands exhibited laboratory profiles significantly different from those of their GC-negative counterparts. Long-term follow-up of these patients will be necessary to determine whether these laboratory abnormalities are predictive of clinical outcomes.
Adult ; Autoantibodies/blood ; C-Reactive Protein/analysis ; Demography ; Female ; Germinal Center/*pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Receptors, Complement 3d/metabolism ; Retrospective Studies ; Salivary Glands, Minor/*pathology ; Sjogren's Syndrome/immunology/metabolism/*pathology

We investigated the clinical and biological significance of germinal centers (GC) present in the minor salivary glands of patients with Sjogren's syndrome (SS). Minor salivary gland tissue biopsies from 93 patients with SS were used to identify GC-like structures, which were confirmed by CD21-positive follicular dendritic cell networks. Patients were compared based upon sociodemographics, glandular and extraglandular manifestations, and laboratory findings including autoantibody profiles, complement, and immunoglobulin levels; EULAR SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. GC-like structures were observed in 28 of 93 SS patients (30.1%). Mean focus scores and CRP levels were significantly higher in GC-positive patients than in GC-negative patients; GC-positive patients also exhibit a higher prevalence of rheumatoid factor and anti-SS-A/Ro antibodies compared to GC-negative patients. No differences in glandular or extra-glandular manifestations were evident between groups. In conclusion, SS patients with GC-like structures in the minor salivary glands exhibited laboratory profiles significantly different from those of their GC-negative counterparts. Long-term follow-up of these patients will be necessary to determine whether these laboratory abnormalities are predictive of clinical outcomes.
Adult ; Autoantibodies/blood ; C-Reactive Protein/analysis ; Demography ; Female ; Germinal Center/*pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Receptors, Complement 3d/metabolism ; Retrospective Studies ; Salivary Glands, Minor/*pathology ; Sjogren's Syndrome/immunology/metabolism/*pathology

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Dendritic Cell Sarcoma, Follicular ; drug therapy ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Head and Neck Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lung Neoplasms ; drug therapy ; secondary ; Male ; Melanoma ; metabolism ; pathology ; Middle Aged ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Vincristine ; therapeutic use