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

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

This study was purposed to verify the binding part of human complement C3 to complement receptor III (CRIII) in monocytes, the peptide rC3B, including the binding-site, was expressed, purified and identified. rC3B, the binding part of human complement C3 to CRIII, was selected by computer-aided modeling and summarizing researches published. Then, rC3B gene fragment was amplified by PCR, and cloned into prokaryotic vector pQE30a. The fusion protein rC3B was expressed in E.coli M15 and purified by Ni(2+)-chelating affinity chromatography. The activity of rC3B was identified by Western blot and adherence assay with monocytes. The results showed that rC3B fragment was obtained, and a prokaryotic expression vector pQE30-rC3B was constructed. rC3B was efficiently expressed and purified. In Western blot, the target protein showed the activity of binding with C3 antibody, while the purified protein showed the activity of adherence with monocytes. It is concluded that the recombinant C3B was obtained and identified, and this study lay the basis for the further functional analysis of C3.
Cloning, Molecular ; Complement C3 ; genetics ; metabolism ; Escherichia coli ; genetics ; metabolism ; Genetic Vectors ; Humans ; Macrophage-1 Antigen ; genetics ; metabolism ; Receptors, Complement 3b ; biosynthesis ; genetics ; isolation & purification ; Recombinant Proteins ; biosynthesis ; genetics ; isolation & purification

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

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

B cell activated co-receptor plays important roles in linkage of innate and acquired humoral immune responses. CD21 molecule in the co-receptor complex is a receptor for C3dg and CD19 molecule enhances BCR signal transduction. CD21 also expresses on the surface of follicular dendritic cells, which mediates the long-term maintenance of antigens and is indispensable for maintaining the memory of B cells. B cell activated co-receptor also has an effect on the negative selection of B cells reactive to autoantigens.
Animals ; Antigens, CD19 ; immunology ; Autoantigens ; immunology ; B-Lymphocytes ; immunology ; Humans ; Receptors, Complement 3d ; immunology ; Receptors, Immunologic

OBJECTIVETo investigate the clinicopathologic features and differential diagnostic methods for follicular dendritic cell sarcoma.

METHODSHistological and immunohistochemical examinations and EBER in situ hybridization were used to investigate the pathological features of 5 cases of follicular dendritic cell sarcoma, and related literature was reviewed.

RESULTSThere were 3 males and 2 females with a median age of 54 years (range, 28 - 75 years). The location of lesions included lymph node (2 cases), tonsil (1 case), stomach (1 case), and liver (1 case). The growth patterns were fascicular or whorls and/or diffuse. The neoplastic cells were spindle or ovoid in shape with indistinct border and slightly eosinophilic cytoplasm. The nuclei were round, oval or spindle in shape with small distinct nucleoli. Warthin-Finkeldey-like multinucleated giant cells were detected in two cases. Mitotic figures were found in 1-22/10 HPF. Immunohistochemical staining showed that CD21 and CD23 (3 of 5), CD35 (4 of 5), D2-40 (4 of 4), and CXCL13 (3 of 4) were positive in neoplastic cells. EBER was detected in one of five cases by in situ hybridization. Four cases were followed-up for 6 approximately 25 months and no recurrence or death was observed yet.

CONCLUSIONFollicular dendritic cell sarcoma is an extremely rare and should be considered as a moderately malignant tumor, and may present histological polymorphism with certain distinctive features. Immunohistochemistry is necessary in differential diagnosis to distinguish from other tumors.

Adult ; Aged ; Antibodies, Monoclonal ; metabolism ; Antibodies, Monoclonal, Murine-Derived ; Chemokine CXCL13 ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Granuloma, Plasma Cell ; metabolism ; pathology ; Humans ; Liver Neoplasms ; metabolism ; pathology ; surgery ; Lymph Nodes ; metabolism ; pathology ; Male ; Membrane Glycoproteins ; metabolism ; Middle Aged ; RNA-Binding Proteins ; metabolism ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Ribosomal Proteins ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo study the cause of low immunologic function and insufficiency of immunoglobulin synthesis in neonates by detecting CD21 expression in B lymphocytes in different age group children.

METHODSThis study consisted of three age group children: 2-26 days (n=18), 6 months-2 years (n=12) and 3-12 years (n=17). CD21 expression in B lymphocytes was detected with flow cytometry. Serum levels of immunoglobulins were measured by immunoturbidimetry.

RESULTSThe percentage and the number of B lymphocytes expressing CD21 in the neonate group were significantly lower than in the other two age groups. The neonate group also showed lower mean fluorescence intension (MFI) of CD21. The percentage and the number of B lymphocytes expressing CD21 as well as the MFI of CD21 increased significantly with the age. The serum levels of IgA and IgM in the neonate group were noticeably lower than those in the other two age groups. The serum levels of IgA and IgM also increased significantly with the age.

CONCLUSIONSLow CD21 expression in B lymphocytes may be related to low function of humoral immunity in neonates.

Adolescent ; Adult ; Age Factors ; B-Lymphocytes ; chemistry ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Receptors, Complement 3d ; blood

Animals ; Beverages ; Macrophages ; drug effects ; metabolism ; Mice ; Mice, Inbred Strains ; Receptors, Complement 3b ; metabolism