Cases reported "Herpesviridae Infections"

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11/157. Fatal herpesvirus-6 encephalitis in a recipient of a T-cell-depleted peripheral blood stem cell transplant from a 3-loci mismatched related donor.

    Human herpesvirus-6 (HHV-6), like all the other herpes viruses, remains latent in host cells after primary infection but can be reactivated in immunocompromised patients causing fever, skin rash, bone marrow (BM) suppression, pneumonitis, sinusitis and meningoencephalitis. We describe the case of a man with chronic myelogenous leukemia who developed encephalitis associated with acute graft-versus-host disease two months after a T-cell-depleted mismatched peripheral blood stem cell transplant. Magnetic resonance images of the brain revealed multiple bilateral foci of signal abnormality. HHV-6 was the only pathogen detected in cerebrospinal fluid by PCR. Treatment with both ganciclovir and foscarnet was unsuccessful and the patient gradually deteriorated and died. Other cases of HHV-6 encephalitis after bone marrow transplantation are reviewed.
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12/157. Retiform hemangioendothelioma: another tumor associated with human herpesvirus type 8?

    Retiform hemangioendothelioma is a rare low-grade angiosarcoma of the skin. It shares some clinical characteristics with Kaposi's sarcoma, a tumor with known human herpesvirus 8 (HHV-8) association. We report a case of retiform hemangioendothelioma in which we detected HHV-8 dna sequences.
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13/157. Successful treatment of human herpesvirus-6 encephalitis after bone marrow transplantation.

    We report two cases of human herpesvirus-6 (HHV-6)-associated encephalitis in patients after BMT. Both patients reported distinct neurological symptoms with disorientation, sleepiness and loss of short-term memory. diagnosis was based on PCR analysis of the cerebrospinal fluid (CSF) positive for HHV-6 variant B-dna. After institution of therapy with foscarnet in both cases, neurological symptoms improved and in one patient clearance of HHV-6-dna from CSF was demonstrated. These cases show that HHV-6 infection has to be considered in patients with neurological symptoms following BMT and effective treatment of HHV-6 encephalitis is possible if instituted early.
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14/157. in situ hybridization detection of human herpesvirus 6 in brain tissue from fatal encephalitis.

    A 23-month-old girl died after 2 days' illness with rash, fever, and convulsions. Neuropathologic findings were consistent with viral hemorrhagic encephalitis in pontine tegmentum and medial thalamic areas. Human herpesvirus 6 (HHV-6) dna was detected in pontine nuclei by in situ hybridization. In addition, polymerase chain reaction for HHV-6 of serum and paraffin-embedded pontine tissue was positive, and serology indicated an acute primary infection. This is the first case showing HHV-6 dna in the brain cells of an immunocompetent patient with acute encephalitis.
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15/157. Kaposi's sarcoma herpesvirus-associated Castleman's disease with poems syndrome.

    The pathogenic link between POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes, and various other clinical signs) syndrome and Castleman's disease is unclear. Roles for M protein in poems syndrome and cytokines in systemic manifestations of multicentric Castleman's disease have been suggested. Recently, pathogenic roles of cytokines in poems syndrome and Kaposi's sarcoma-associated herpesvirus (KSHV) in Castleman's disease have been reported. We report on a patient with KSHV-associated Castleman's disease with poems syndrome, and suggest a possible role of KSHV in initiating and linking these two diseases.
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keywords = herpesvirus
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16/157. HLA-mismatched CD34-selected stem cell transplant complicated by HHV-6 reactivation in the central nervous system.

    We report here a patient who suffered from PCR- confirmed human herpesvirus type 6 (HHV-6) meningoencephalitis after allogeneic purified CD34 cell transplantation from his HLA-mismatched sibling donor, even though he had been on intense prophylaxis with i.v. ganciclovir (GCV), acyclovir (ACV) and gamma-globulin containing a specific antibody against HHV-6. Serological evaluation disclosed that both the donor and recipient had IgG antibody against HHV-6 before transplantation. His blood WBC count started to transiently increase on day 10, and all blood components had decreased by day 20. He then developed a severe headache and high blood pressure, and sporadic abnormal neurological findings including nystagmus and delirium. An analysis of cerebrospinal fluid (CSF) revealed 8 cells/microl, a glucose level of 130 mg/dl and a protein level of 201 mg/dl (normal, 50 mg/dl) on day 26. At the time, HHV-6 was detected only in CSF by a PCR-based method and he was diagnosed as having meningoencephalitis due to the local reactivation of HHV-6. Although he failed to respond to high-dose therapy with ACV (60 mg/kg/day) and gamma-globulin, the dna of this virus disappeared from the CNS upon treatment with GCV (30 mg/kg/day) combined with the intraventricular infusion of alpha-interferon. His clinical course was further complicated with meningoencephalitis due to staphylococcus epidermidis, and he died of tentorial herniation on day 79 without the recovery of blood components. This experience may indicate that intense prophylaxis to prevent reactivation of HHV-6 in the CNS is essential for the management of such profoundly immunosuppressed patients.
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17/157. Multinucleate cell angiohistiocytoma: report of two cases with no evidence of human herpesvirus-8 infection.

    Multinucleate cell angiohistiocytoma (MCA) is a vascular tumor of unknown pathogenesis. Possible misinterpretation of this disorder with Kaposi's sarcoma (KS), a human herpesvirus-8 (HHV-8)-associated tumor, prompted us to look for this virus in two women with MCA. None of the multiple skin specimens obtained from both our patients produced amplified HHV-8 dna. Using a cell culture methodology similar to that used for KS, we established cell cultures from MCA lesions. While KS spindle cells are known to exhibit in vitro invasive properties and can be grown up to more than 20 passages, the MCA-derived cells were short-lived and were not able to traverse basement membranes. Taken together, our data support the hypothesis that MCA is not a neoplasm but a benign vascular proliferation which is clearly distinguishable from KS.
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18/157. Human herpesvirus-6 associated encephalitis with subsequent infantile spasms and cerebellar astrocytoma.

    A 14-month-old girl presented after 3 days of fever, floppiness, and diffuse urticarial exanthem. She developed encephalitis and carditis and 1 week later, intractable seizures. Initial CT and MRI showed no changes in the brain parenchyma. On days 14 and 34 after the onset of symptoms, a human herpesvirus-6 (HHV-6) genome in cerebrospinal fluid was identified by polymerase chain reaction (PCR). Convulsions became more frequent and 11 weeks from the onset, they changed to typical infantile spasms with hypsarrhythmic electroencephalogram. She gradually lost her social contact and ability to walk and sit. Eleven months after the primary infection, a repeated MRI of the brain revealed a cystic tumour of 2 cm in diameter near the vermis. The tumour was surgically removed, and shown to be a pilocytic astrocytoma on histopathological examination. HHV-6 dna was detected by PCR in new tumour tissue. This is the first reported case of HHV-6 encephalitis associated with carditis, infantile spasms, and a subsequent brain tumour containing the HHV-6 genome.
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keywords = herpesvirus
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19/157. Herpesvirus dna detection in cerebral spinal fluid: differences in clinical presentation between alpha-, beta-, and gamma-herpesviruses.

    To evaluate the role of 6 human herpesviruses (cytomegalovirus (CMV), Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2 and varicella zoster virus (VZV)) in infections of the nervous system, cerebrospinal fluid (CSF) samples from 662 patients with suspected viral aetiology to neurological symptoms were investigated for presence of herpesviral dna in a PCR-based study. Of the 69 patients (2 patients had 2 herpesvirus dna detected in CSF) who had herpesvirus dna detected in the CSF, 60 (87%) were non-immunocompromised (CMV 7; HHV-6 6; EBV 16; HSV-1 18; HSV-2 9 and VZV 6) and 9 (13%) were immunocompromised (CMV 3; HHV-6 0; EBV 5; HSV-1 0; HSV-2 1 and VZV 0). The study was performed in a retrospective/prospective manner. The HSV-1, HSV-2, VZV and CMV dna-positive patients usually had typical clinical syndromes, such as encephalitis/myelitis and meningitis, but also other neurological conditions were associated with findings of these viruses. HHV-6 and EBV dna were detected in patients presenting with a variety of neurological symptoms, and in some of the cases, concurrent with diagnosis of other infections of the central nervous system. Despite the overall variability of clinical conditions seen, a pattern associated with each investigated herpesvirus was discernable as regards clinical presentation.
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ranking = 1.8
keywords = herpesvirus
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20/157. Human herpesvirus-6 (HHV-6)-associated hemophagocytic syndrome.

    Virus-associated hemophagocytic syndrome (VAHS) is characterized by histiocytic proliferation and phagocytosis triggered by virus infections. viruses in the herpes group, especially the Epstein-Barr virus (EBV), are well known to cause VAHS; however, the relationship between this syndrome and human herpesvirus-6 (HHV-6) infection has rarely been reported. In this study, we describe a 23-month-old girl who exhibited typical manifestations of VAHS associated with HHV-6 infection. To the best of our knowledge, this case is the fifth reported case in the English literature.
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