1/82. 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 = organ (Clic here for more details about this article) |
2/82. Subacute sensory neuropathy associated with Epstein-Barr virus.A 35-year-old man experienced severe sensory loss, pseudoathetosis, and areflexia during recovery from a severe viral illness. Sensory nerve action potentials were absent, motor conduction velocities were mildly slowed, and blink reflexes were normal. magnetic resonance imaging (MRI) revealed abnormal signal within the central and dorsal aspects of the thoracic cord. Acute and convalescent Epstein-Barr virus (EBV) titers suggested EBV as the etiology. Subacute sensory neuropathy, with peripheral and central nervous system involvement, is a rare complication of EBV infection.- - - - - - - - - - ranking = 0.029695894245662keywords = nerve (Clic here for more details about this article) |
3/82. Extracorporeal photochemotherapy for Epstein-Barr virus-associated lymphoma after lung transplantation.Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication after transplantation of solid organs. Highest incidence rates have been reported for lung transplant recipients. With the current treatment strategy for early onset PTLD, a reduction of immunosuppressive drugs, mortality of lung transplant recipients with PTLD remains high, due to both, incomplete control of PTLD and transplant rejection. We present a lung transplant recipient with a history of acute rejection and Epstein Barr virus-associated posttransplantation malignant non-Hodgkin's lymphoma. Extracorporeal photochemotherapy, in combination with a moderate reduction of immunosuppressive therapy, resulted in complete disappearance of PTLD. After a first year of follow-up, no further rejection and no recurrence of PTLD have occurred. Treatment with ECP, with its beneficial effects on both, rejection after organ transplantation and malignant lymphoma, may be a particularly valuable approach for the treatment of PTLD in patients after lung transplantation, with its increased risk for transplant rejection.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/82. 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 = 0.5keywords = organ (Clic here for more details about this article) |
5/82. Paediatric chiasmal neuritis--typical of post-Epstein-Barr virus infection?A case of chiasmal neuritis with bilateral, asymmetric optic nerve involvement is presented. association with recent Epstein-Barr virus infection is suggested. The clinical picture, possible aetiological factors, and causative relation to Epstein-Barr virus are presented.- - - - - - - - - - ranking = 0.029695894245662keywords = nerve (Clic here for more details about this article) |
6/82. Hemophagocytic syndrome in pregnancy.BACKGROUND: Hemophagocytic syndrome is characterized by nonmalignant histiocytes that undergo uncontrolled phagocytosis of normal hematopoietic cells. Clinical severity ranges from complete recovery to rapid deterioration and death. CASE: thrombocytopenia was discovered upon routine initial prenatal evaluation of a 24-year-old, gravida 2, para 1, at 29 weeks' gestation with a history of necrotizing lymphadenitis. Cytopenia and elevated transaminases developed, followed by hyperpyrexia. The patient delivered and her postpartum course was complicated by coagulopathy, multiorgan failure, and death. Bone marrow biopsy confirmed hemophagocytic syndrome. CONCLUSION: early diagnosis of hemophagocytic syndrome during pregnancy might be helped by recognizing symptoms and signs, including a history of necrotizing lymphadenitis, and obtaining a bone marrow biopsy.- - - - - - - - - - ranking = 0.5keywords = organ (Clic here for more details about this article) |
7/82. Use of radiation therapy in posttransplant lymphoproliferative disorder (PTLD) after liver transplantation.Posttransplant lymphoproliferative disorder (PTLD) is a common and life-threatening complication of immunosuppression used to prevent rejection of solid organ and bone marrow transplants. There is no standardized treatment algorithm, but numerous management strategies are available. We describe a patient who developed a solitary lymphoproliferative lesion in the porta hepatis 9 months after orthotopic liver transplant. Following reduction in immunosuppression with no response, she was treated with involved field radiotherapy utilizing CT-based treatment planning. A partial radiographic response was obtained, and she has not developed disease in the engrafted liver or systemically. Based on the present case report, involved field radiotherapy seems to be a reasonable treatment option for patients with localized PTLD. Int. J. Cancer (Radiat. Oncol. Invest.) 90:104-109, 2000.- - - - - - - - - - ranking = 0.5keywords = organ (Clic here for more details about this article) |
8/82. Development of a biologically distinct EBV-related lymphoproliferative disorder following autologous bone marrow transplantation for an EBV-negative post-renal allograft Burkitt's lymphoma.Post-transplant lymphoproliferative disorder (PTLD) is a known complication of both solid organ transplantation and allogeneic bone marrow transplantation (BMT) but is rarely seen following autologous BMT. We report the case of a 45 year-old female who developed Burkitt's lymphoma eight years after a renal allograft. This PTLD was found to have lambda light chain restriction, contained del(8)(q24) and add(14)(q32), and was negative for EBV on immunohistochemical and dna-based PCR analyses. Immunoglobulin heavy chain (IgH) PCR studies revealed a prominent clonal rearrangement. She responded to intravenous cyclophosphamide and proceeded to high-dose chemoradiotherapy and mafosfamide-purged autologous BMT. Thirty-nine days post-BMT she presented with cough and fever and developed hepatic dysfunction; abnormal lymphoplasmacytoid cells were noted in the peripheral blood. Investigations revealed kappa light chain restriction, an oligoclonal IgH rearrangement, a normal karyotype and PCR studies for EBV were positive, consistent with a clinically and biologically distinct PTLD. She initially improved following discontinuation of immunosuppression, but then deteriorated abruptly and died 58 days post-BMT. It is likely that the two separate episodes of PTLD in this patient, one of which was atypical, arose as a result of both the chronic use of cyclosporine and the impairment of cell-mediated immunity associated with autologous BMT. The sequence of events in this patient should contribute to a better understanding of late-onset, EBV-negative PTLD.- - - - - - - - - - ranking = 0.5keywords = organ (Clic here for more details about this article) |
9/82. Verotoxin targets lymphoma infiltrates of patients with post-transplant lymphoproliferative disease.Post-transplant lymphoproliferative disease (PTLD) is an invasive, EBV expressing B lymphoma and a major cause of morbidity and mortality following organ transplantation. Presently there is limited therapy available; rather the patient often loses the allograft or succumbs to the malignancy. CD77 (or globotriaosyl ceramide -Gb(3)) is a germinal center B cell marker [Gregory et al. Int J Cancer 1998;42:213-20; Gregory et al., J Immunol 1987;139:313-8; Mangeney et al. Eur J Immunol 1991;21:1131-40], expressed on most EBV infected B cells and is the receptor for the E. coli derived verotoxin (VT) [Lingwood CA. Advances in Lipid research 1993;25:189-212]. We present the basis of a possible novel approach to PTLD therapy utilizing the specific targeting of VT to the infiltrating lymphoma cells. Biopsies of adenoid, kidney or liver tissue of four PTLD patients were stained with verotoxin to determine expression of CD77. VT is a potent inducer of necrosis/apoptosis of receptor positive cells. In each PTLD case, the infiltrating EBV positive B lymphoma cells were strongly and selectively stained with VT, identifying CD77 as a new marker for these cells. For such individuals, VT might provide the basis of an approach to control their malignancy.- - - - - - - - - - ranking = 0.5keywords = organ (Clic here for more details about this article) |
10/82. Lymphomatous features of aggressive NK cell leukaemia/lymphoma with massive necrosis, haemophagocytosis and EB virus infection.AIMS : Aggressive natural killer (NK) cell leukaemia will be categorized as a distinct entity in the new WHO classification of malignant lymphomas. However, its non-leukaemic features remain unclear. We therefore investigated the morphological and immunophenotypic features of this lymphoma. methods AND RESULTS : Four cases with aggressive NK cell lymphoma were morphologically and immunohistochemically studied. All cases followed an aggressive course with death occurring within about 3 months of initial presentation. In these cases, the neoplastic cells disseminated throughout systemic lymph nodes and invaded various tissues and organs. The lymphoma cells were large cells showing nuclear irregularity and a pattern of sinusoidal invasion in lymph nodes. apoptosis and coagulation necrosis were both frequently observed. Haemophagocytosis was observed in all cases. Neoplastic cells in paraffin-embedded tissue specimens from these patients had CD3(CD3epsilon) CD56(123C3) granzyme TIA-1 EBERT CD43(MT1)- CD45RO(UCHL-1)- CD57(Leu7)- CD20(L26)- phenotypes. In the two cases where tissue was available for immunohistochemical study in frozen sections, neoplastic cells showed CD56(Leu19) perforin Fas ligand(FasL) CD2(Leu5b)- CD3(Leu4)- CD4(Leu3)- CD5(Leu1)- CD7(Leu9)- CD8(Leu2)- betaF1- TCRdelta1- phenotypes. CD16(Leu11b) was positive in one case. CONCLUSIONS: : Natural killer cell lymphomas appear to represent a non-leukaemic counterpart of aggressive natural killer cell leukaemia, a relationship similar to that in adult T-cell leukaemia/lymphoma. awareness and diagnosis of this aggressive lymphoma is important because of its fulminant course.- - - - - - - - - - ranking = 0.5keywords = organ (Clic here for more details about this article) |
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