1/118. Systemic granulomatous arteritis associated with Epstein-Barr virus infection.A 61-year-old woman initially presented with symptoms and findings reminiscent of infectious mononucleosis, and her illness then took a rapidly fatal course. autopsy revealed widespread granulomatous arteritis, with multinucleated giant cells but without eosinophils and fibrinoid necrosis, affecting small arteries and arterioles and infiltration of haemophagocytic histiocytes into many organs. in situ hybridization with Epstein-Barr virus (EBV)-specific oligonucleotide probes showed positive signals in the infiltrating immune cells and epithelial and endothelial cells of the affected organs. EBV-associated haemophagocytic syndrome (EBV-AHS) with systemic granulomatous arteritis was diagnosed. From the immunophenotypes of the infiltrating immune cells, a possible role of CD4 T-cells in the pathogenesis of this haemophagocytic syndrome and granulomatous vasculitis was suggested.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/118. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/118. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.OBJECTIVE: Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues. methods: A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American lymphoma (REAL) classification. The clinical and follow-up data were collected. RESULTS: The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3 , CD30 , and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3 , CD56 , and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively. CONCLUSION: This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/118. EBV infection induced transformation of benign T lymphoproliferative state in patient with chronic active EBV infection into malignant lymphoma: implication of EBV infection as additive oncogenic factor in tumorigenesis.An 11-year-old girl with chronic EBV (Epstein-Barr virus) infection, who later developed malignant lymphoma in the lung, is reported. She had an increased number of V alpha2, V beta8, CD3, CD4, and HLADR positive activated lymphocytes (20-30% of total lymphocytes) in peripheral blood. One year later, she developed lymphoma in the lung, which was V alpha2, V beta8, CD3, CD4, HLADR and IL2Rbeta positive. At that time, the population in the peripheral blood increased up to 40%, but there was no evidence of lymphoma in the bone marrow. in situ hybridization revealed lymphoma cells were EBER-1 positive but gp350/220 and LMP mRNA negative. The EBV genome was detected in the tumor, but not in the peripheral T cells. Clonal analysis of the lymphoma cells revealed monoclonal rearrangement of the TcRbeta and gamma gene, however, investigation of the terminal repeat of EBV gene did not show the monoclonal pattern. These results indicate that infection of EBV into clonally activated T cells was related with transformation from benign lymphoproliferative disease to malignant lymphoma in this patient.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/118. Case report of lymphoepithelioma-like carcinoma of the lung--lymphoid population consisting of cytotoxic T cells in resting state.The present report describes a case of lymphoepithelioma-like carcinoma (LELC) of the lung and presents immunohistochemical and in situ hybridization (ISH) studies of the tumor. A 39-year-old Chinese woman, who was born in china and emigrated to japan at the age of 29, suffered from a cough for 2 years and received a middle and lower lobectomy with mediastinal lymph node dissection after induction chemotherapy. The tumor consisted of undifferentiated carcinoma and areas of more differentiated squamous cell carcinoma with an intense lymphoid infiltrate. Serological studies and ISH studies showed EBV infection of the tumor. The immunophenotype of tumor-infiltrating t-lymphocytes (TITL) of the present case was examined immunohistochemically and was compared with that of an LELC case reported previously. Most CD3-positive T cells of TITL in both cases were labeled with both CD8 and TIA-1 but not with granzyme-B, indicating the TITL to be cytotoxic T lymphocytes (CTL) in the resting state. The lack of CTL activation at the tumor site might have been due to local inhibition of EBV-specific CTL responses such as T-cell anergy. Because the EBV-specific CTL derived from peripheral blood lymphocytes, in contrast to the TITL, may not be influenced by either tumor-produced suppressor factors or negative regulatory T cells, they may inhibit the hematogenous metastasis of EBV-positive LELC, possibly resulting in a better prognosis. Because LELC of the lung responded to preoperative chemotherapy in the present study, it may be useful for reducing the local tumor burden and facilitate subsequent local therapy, although the mechanism of chemosensitivity of LELC remains unknown.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/118. intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection.We report the association of neurological and intestinal disorders with the reactivation of Epstein-Barr virus (EBV) in a child. This previously healthy 13-yr-old boy presented with pharyngitis and acute abdominal ileus. laparotomy excluded a mechanical obstruction. Postoperatively, he suffered from prolonged intestinal obstruction, pandysautonomia, and encephalomyelitis. Histological examination of the appendix and a rectal biopsy taken 3 months after the onset showed an absence of ganglion cells (appendix) and hypoganglionosis (rectum), with a mononucleate inflammatory infiltrate in close contact with the myenteric neural plexuses. EBV-PCR was positive in the blood and cerebrospinal fluid, and in situ hybridization with the Epstein-Barr virus encoded rna probe showed positive cells throughout the appendix wall including the myenteric area, in a mesenteric lymph node, and in the gastric biopsies. EBV spontaneous lymphocytic proliferation was noted in the blood. The serology for EBV showed previous infection but anti-early antigen antibodies were present. No immunodeficiency was found. Neurological and GI recovery occurred after 6 months of parenteral nutrition and bethanechol. The omnipresence of EBV associated with the neurointestinal symptoms suggest that the virus was the causal agent. This is the first documented case of acquired hypoganglionnosis due to EBV reactivation.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/118. Vanishing bile duct syndrome associated with chronic EBV infection.We reported here an adult patient with vanishing bile duct syndrome due to chronic EBV infection. A 22-year-old male was admitted to a nearby hospital complaining of a sore throat and jaundice. He received a high dose of prednisolone for bile stasis of acute viral hepatitis. However, the hepatitis did not improve, and he was transferred to our hospital. He had exhibited jaundice for one year as well as hemophagocytic syndrome and intestinal perforation. Subtotal intestinal resection was successfully performed. Three follow-up biopsied liver specimens indicated vanishing bile duct syndrome. Positive results of EBV-dna in his serum and mRNA of EBV by in situ hybridization of his liver indicated that massive doses of prednisolone caused chronic EBV infection and vanishing bile duct syndrome.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/118. Late Epstein-Barr virus infection of a hepatosplenic gamma delta T-cell lymphoma arising in a kidney transplant recipient.BACKGROUND AND OBJECTIVE: gd T-cell lymphomas are only exceptionally observed in transplanted patients. Aim of this study was the detailed characterization of one such case. DESIGN AND methods: The patient developed spontaneous splenic rupture six years after kidney transplantation. The splenic red pulp was infiltrated by medium-sized and large lymphoid cells with two or more nucleoli. At autopsy, similar lymphoid cells infiltrated the hepatic sinusoids. Histologic, immunologic and molecular studies were carried out. RESULTS: By immunohistochemistry, the atypical lymphoid cells were found to express CD3, CD45 and CD43, indicating their T-lineage origin. Approximately 99% of spleen mononuclear cells (MNC) were CD3( ), gammadelta TcR , CD4-, CD8-, alphabeta TcR-. A clonal gammadelta TcR rearrangement (Vgamma1-Jgamma1.3/2.3-Cgamma2; Vdelta1-Ddelta2-Jdelta1) was detected. The final diagnosis was peripheral T-cell lymphoma, hepato-splenic gammadelta-type. EBV infection of spleen MNC was documented by molecular studies. However, in situ hybridization for EBER-1 (EBV-rna) showed that only a minority of malignant lymphoid cells (5-7%) were EBV-infected. INTERPRETATION AND CONCLUSIONS: It is concluded that EBV infection was as a late event involving an already transformed gd T-cell clone.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
9/118. Detection of Epstein-Barr virus in posttransplantation T cell lymphoma in a kidney transplant recipient: case report and review.Posttransplantation T cell lymphomas (PTTLs) are rather unusual, and their etiology remains unclear. We describe a case of Epstein-Barr virus (EBV)-associated small bowel T cell lymphoma in a patient 5 years after kidney transplantation. EBV was detected in a biopsy sample by in situ hybridization, immunohistochemical staining, and polymerase chain reaction analysis. Eight previously reported cases of EBV-associated PTTL are reviewed, in which special attention is paid to the methods used for assessing EBV. This case of EBV-associated PTTL is believed to be the most completely studied from the point of view of the methods used for detection of EBV. The prognosis of PTTL is poor, but it has been reported that therapeutic approaches can be successful if they are given early in the course of the illness. Therefore, it is necessary to improve the diagnosis PTTL and to assess the precise involvement of EBV in posttransplantation lymphoproliferative disorders.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
10/118. central nervous system involvement of Epstein-Barr virus lymphoproliferative disease in a patient with acute lymphocytic leukemia.Epstein-Barr virus-related lymphoproliferative disease (EBV-LPD) is a serious and often fatal complication of a variety of immune-suppressed conditions. A 6-year-old boy undergoing chemotherapy for standard-risk acute lymphocytic leukemia experienced separate episodes of EBV-LPD in different organ systems. The patient experienced three separate episodes of EBV-LPD in the cervical lymph node, the central nervous system (CNS), and the liver occurring, respectively, in January 1992, February 1992, and November 1993 after the completion of chemotherapy in May 1993. The EBV presence was confirmed by in situ hybridization in the biopsy samples from each lesion. Several different treatment modalities, including acyclovir, intravenous gamma globulin, and surgery were used to combat the EBV-LPD. The patient has recovered completely, with normal CNS and liver function, and for the past 6 years has experienced leukemia remission while not receiving chemotherapy. Careful monitoring of patients and the use of new immune therapies offer the highest chance for successful outcomes in such patients.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
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