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1/13. Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus.

    We describe a patient with longstanding systemic lupus erythematosus (SLE) in remission who presented with recent onset neurological symptoms. magnetic resonance imaging, followed by a brain biopsy and in situ hybridization, confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML). The clinical findings in this patient emphasize the importance of considering PML in an individual with SLE and neurological abnormalities.
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2/13. Necrotizing ileitis caused by cytomegalovirus in patient with systemic lupus erythematosus: case report.

    We report a systemic lupus erythematosus (SLE) patient with necrotizing ileitis diagnosed at a tertially care centre in thailand. The patient was surgically explored because peritonitis was suspected and segmental gangrenous and perforation of the terminal iliem were found. The pathological finding was necrotizing ileitis with appearance of cytomegalic intranuclear inclusion body. The presence of cytomegalovirus (CMV) infection in tissue was confirmed by CMV-dna detection using polymerase chain reaction and ELISA probe hybridization method. The hemoculture and peritoneal fluid culture results revealed no pathogenic organisms. Postoperatively, the clinical course of the patient deteriorated and she developed hypotension. Vasopressive drugs were administered without clinical improvement. She expired on day 5 postoperation. Regarding CMV infection, the organism involves the small bowel in only 4.3 per cent of all CMV infections of the gastrointestinal tract. Isolated cases of ileal perforation due to CMV infection have never been reported in a SLE patient. Thus, chronic right lower abdominal pain, fever with or without diarrhea in immunocompromised patients should cause clinicians to consider CMV ileitis in the differential diagnosis. Immediate surgical resection and prompt antiviral therapy lead to successful treatment.
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3/13. Microchimerism in a female patient with systemic lupus erythematosus.

    Systemic lupus erythematosus (SLE) is a serious multisystem disease that has a striking propensity to affect women. The cause of SLE remains elusive. Fetomaternal cell trafficking, or the passage of fetal cells into the maternal circulation, is now a well-established phenomenon. In addition, fetal cells have been implicated in the development of preeclampsia and in the pathogenesis of scleroderma. We undertook this study to determine whether fetomaternal cell trafficking might also be involved in pathogenic processes in SLE. fluorescence in situ hybridization analysis was performed using X and y chromosome-specific probes on affected and unaffected tissue obtained at autopsy from a woman who had previously given birth to 2 males and who had died of complications of SLE. The goal of the analysis was to detect the presence of male cells of putative fetal origin. Male cells were found in every histologically abnormal tissue type that was examined, but were not found in histologically normal tissue. These data suggest that fetal cells may be associated with SLE. It is unclear whether their presence may be related to disease causation, an effect of disease progression, or unrelated to disease pathology. However, this case study is an important step toward understanding the potential relationship between fetomaternal cell trafficking and SLE pathology.
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4/13. Lymphocytic interstitial pneumonitis associated with Epstein-Barr virus in Systemic Lupus Erythematosus and sjogren's syndrome. Complete remission with corticosteriod and cyclophosphamide.

    Lymphocytic interstitial pneumonitis (lip) is characterized by diffuse bilateral pulmonary infiltrations in both lower lobes. Pleomorphic lymphohistiocytes including mature lymphocytes, variable admixture of plasma cells and other mononuclear cells infiltrate within the pulmonary interstitium, ranging from widened septa to confluent masses. We report a case of lip associated with Epstein-Barr virus in a patient with SLE and sjogren's syndrome. A 50-year-old woman was admitted with insidious onset of progressive dyspnea for 20 days. She suffered from arthritis 10 years earlier without specific diagnosis. A radiography of chest has showed bilateral consolidative infiltrations with pleural effusion in both lower lung fields. Open lung biopsy documented lymphocytic interstitial pneumonitis which expressed Epstein-Barr virus genome in situ hybridization study. Following corticosteroid and cyclophosphamide therapy, clinical symptoms and radiologic infiltrations gradually remitted.
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5/13. Post-transplant T-cell lymphoproliferative disorder/T-cell lymphoma: a report of three cases of T-anaplastic large-cell lymphoma with cutaneous presentation and a review of the literature.

    AIMS: To report the clinical, pathological and immunohistochemical features of three cases of post-transplant T-cell lymphoproliferative disorder (T-PTLD) T-cell lymphoma with primary cutaneous presentation. methods AND RESULTS: Three cases of primary cutaneous post-transplantation anaplastic large-cell lymphomas occurred in renal transplant recipients and were shown to display a T-cell immunophenotype; all were ALK 1 protein and EMA negative and two were Epstein-Barr virus positive using in-situ hybridization. Two displayed a CD4 phenotype, two were focally CD56 and all three were negative for the cytolytic enzyme granzyme B. In two cases monoclonality was established by T-cell receptor gene rearrangement study. All presented with nodular cutaneous involvement and all were ultimately fatal. CONCLUSION: T-PTLDs are uncommon histological subtypes both in a general context and associated with cutaneous presentation. Our findings suggest clinicopathological and immunophenotypic similarities to primary cutaneous anaplastic large-cell lymphoma but with a progressive clinical behaviour similar to previously reported T-PTLD and to systemic nodal ALK- anaplastic large-cell lymphoma.
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6/13. Reactive hyperplasia with giant follicles in lymph node lesions from systemic lupus erythematosus patients. Report of three cases.

    Lymph node enlargement is common in active systemic lupus erythematosus (SLE), a disease that is characterized by well-defined clinical criteria. Histologically, although lymphadenopathy associated with SLE exhibits marked histological diversity and occasionally shows atypical lymphoproliferative disorders, there has not been any description of the histopathological features of reactive lymph node hyperplasia with giant follicles (RHGF). We here report three such cases. The subjects were a 23-year-old Japanese female, a 44-year-old Japanese female and a 49-year-old Japanese male. All three patients initially presented with systemic lymphadenopathy. They also had systemic symptoms and abnormal laboratory findings indicating active disease, although two patients did not fulfill the diagnostic criteria for SLE at lymph node biopsy. Histologically, three lesions were characterized by numerous enlarged, coalescing lymphoid follicles with distortion rather than effacement of the lymph node architecture. By in situ hybridization, Epstein-Barr virus (EBV) genomes were demonstrated in two cases. The present three cases indicate that lymphadenopathy associated with SLE representing RFGH should be differentiated from the early stage of hiv-related lymphadenopathy as well as follicular lymphoma, particularly the floral variant. The authors would like to stress that the RHGF which is described in the present study should be listed in the pathohistology of SLE lymphadenopathy.
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7/13. Squamous cell carcinoma arising in a case of vulvitis granulomatosa or vulval variant of melkersson-rosenthal syndrome.

    A case of vulvitis granulomatosa or the vulval variant of melkersson-rosenthal syndrome (MRS) in a young adult patient with systemic lupus erythematosus is presented. She also had evidence of cervicovulvovaginal human papilloma virus (HPV) infection. Nine years later she developed a squamous cell carcinoma (SCC) of the vulva. HPV 6/11 was found by dna in situ hybridization within a vulval condyloma as well as within the carcinoma. This case is unusual in that SCC associated with HPV 6/11 developed in her vulva affected by MRS rather than in the cervix, where SCC was more likely to occur considering this patient's age.
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8/13. Active human herpesvirus-6 (HHV-6) infection associated with Kikuchi-Fujimoto disease and systemic lupus erythematosus (SLE).

    histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) is a well defined disorder primarily affecting young adults. The cause of this disease is still unknown. The authors report a case of a 37-yar old woman with Kikuchi-Fujimoto disease and systemic lupus erythematosus (SLE). Serologic testing for HHV-6 antibodies revealed an active infection. An excised cervical lymph node contained HHV-6 genome demonstrated by using in situ hybridization. Active HHV-6 infection should be considered in Kikuchi-Fujimoto disease.
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9/13. Manifestations and treatment of human parvovirus B19 infection in immunocompromised patients.

    We diagnosed infections from human parvovirus B19 in three patients by using dot-blot hybridization and a polymerase chain reaction to detect B19 dna and using an enzyme immunoassay to detect IgG and IgM to B19. For 5 months a 5-year-old boy with acute lymphoblastic leukemia in remission had anemia without reticulocytes or bone marrow erythrocyte precursors. His serum lacked IgG and IgM to B19 but contained B19 dna. He received gamma globulin intravenously (0.4 gm/kg/day for 5 days); his viremia promptly cleared and reticulocytosis developed. A 14-year-old boy with acute lymphoblastic leukemia in remission had fever, rash, neutropenia (less than 300 leukocytes/mm3), and a hemophagocytic syndrome lasting 3 weeks. His serum contained IgM to B19 and B19 dna. Without therapy, IgG to B19 developed; although low levels of B19 dna persisted, the leukocyte count returned to normal. In a 19-year-old patient with systemic lupus erythematosus and hemolytic anemia, an aplastic crisis lasted 2 weeks. Her serum lacked IgG and IgM to B19 but contained B19 dna. Without therapy, IgG and IgM to B19 appeared, viremia diminished, and reticulocytosis occurred. These patients illustrate the varied manifestations of chronic B19 infections, the importance of dna detection for diagnosis, and the possible efficacy of gamma globulin therapy.
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10/13. Identification of human immunodeficiency virus hybridizing sequences in the peripheral blood of a patient with systemic lupus erythematosus.

    Systemic lupus erythematosus, a multisystemic disorder, is considered a prototype of the autoimmune diseases. Although its cause remains unknown, a viral etiology has been proposed. We report that a rapid and sensitive messenger rna in situ hybridization technique detected hybridizing sequences to the human immunodeficiency virus type in the peripheral blood cells of a woman with systemic lupus erythematosus in whom the presence of acquired immuno-deficiency syndrome was reasonably excluded.
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