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1/105. The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports.

    A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral dna from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC).
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ranking = 1
keywords = virus, fever
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2/105. Stealth virus epidemic in the Mohave Valley: severe vacuolating encephalopathy in a child presenting with a behavioral disorder.

    An infectious illness, attributed to atypically structured cytopathic "stealth" viruses, occurred in 1996 in the Mohave Valley region of the united states. A stealth virus-infected child from this region has developed a severe noninflammatory, vacuolating (spongiform) en cephalopathy. The illness initially presented as a behavioral problem without overt neurological signs. Extensive investigations, including repeated magnetic resonance imaging, two brain biopsies, and stealth virus cultures, have helped define the disease process occurring in this child. Significant clinical benefit with apparent retardation of disease progression occurred during a 6-week course of ganciclovir therapy. The potential contributing role of stealth virus infections in children presenting with behavioral problems needs to be addressed.
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ranking = 7.9975061933119
keywords = virus
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3/105. Viral infections in interferon-gamma receptor deficiency.

    interferon-gamma receptor deficiency is a recently described immunodeficiency that is associated with onset of severe mycobacterial infections in childhood. We describe the occurrence of symptomatic and often severe viral infections in 4 patients with interferon-gamma receptor deficiency and mycobacterial disease. The viral pathogens included herpes viruses, parainfluenza virus type 3, and respiratory syncytial virus. We conclude that patients with interferon-gamma receptor deficiency and mycobacterial disease have increased susceptibility to some viral pathogens.
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ranking = 2.999064822492
keywords = virus
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4/105. Recurrent viral associated hemophagocytic syndrome in a child with Langerhans cell histiocytosis.

    Langerhans cell histiocytosis (LCH) with subsequent viral-associated hemophagocytic syndrome (VAHS) or secondary hemophagocytic lymphohistiocytosis (HLH) is extremely rare. A 15-month-old girl with disseminated LCH experienced three episodes of VAHS during maintenance therapy. Viral infection, with influenza A, herpes simplex, and adenovirus, respectively, was documented at each episode. She recovered each time after interruption of maintenance therapy. The occurrence of fever and pancytopenia in patients with chemotherapy-treated LCH can be associated with VAHS and not with relapsing LCH.
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ranking = 1
keywords = virus, fever
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5/105. 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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ranking = 2.0103608738725
keywords = virus, encephalitis
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6/105. Papular acrodermatitis of childhood: the Gianotti-Crosti syndrome.

    Papular acrodermatitis of childhood (PAC), also known as Gianotti-Crosti syndrome, is a self-limited disorder with acute onset generalized lymphadenopathy and monomorphic lentil-sized, dense, nonconfluent, symmetric, flat-topped, non-pruritic papules. We describe 2 patients, one with anicteric hepatitis, lymphocytosis, and positive hepatitis b surface antigenemia, and the other with a cytomegalovirus (CMV) infection.
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ranking = 0.99968827416399
keywords = virus
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7/105. Unusual viral infections (progressive multifocal leukoencephalopathy and cytomegalovirus disease) after high-dose chemotherapy with autologous blood stem cell rescue and peritransplantation rituximab.

    Efforts to reduce relapse of non-Hodgkin lymphoma after autologous transplantation have included ex vivo stem cell selection and/or peritransplantation immunotherapy. The late infectious and immunologic consequences of these maneuvers are not well understood, although an increase in early cytomegaloviral disease after CD34( ) stem cell selection and an alteration in immunoglobulin and T-cell recovery after peritransplantation rituximab has been noted. We report the first 2 cases of progressive multifocal leukoencephalopathy caused by JC papovavirus after autologous peripheral blood stem cell transplantation and a case each of cytomegalovirus retinitis and pneumonitis. All 4 patients experienced significant impairment of CD4 T-cell recovery, placing them at risk for these unusual viral infections. The clustering of cases is concerning because all occurred shortly after the introduction of peritransplantation rituximab into treatment protocols (4 of 62 immunotherapy recipients compared with 0 of 276 without; z = 3.595; P <.001), although a direct association with this CD20 B-cell-directed therapy remains speculative.
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ranking = 5.9981296449839
keywords = virus
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8/105. Increased serum thymidine kinase activity in acute sarcoidosis.

    This is the first case report of acute sarcoidosis with increased serum thymidine kinase (TK) activity. A 43-year-old male presented fever, swelling of parotid glands, lymphadenopathy, and peripheral neuropathy. sarcoidosis was pathologically diagnosed by lung and parotid gland biopsy. His serum TK, which was increased to 11.2 U/l at diagnosis (normal <5 U/l), normalized after glucocorticoid therapy. serum TK has been considered as a good marker of the proliferative activity of various types of neoplasms. Its rise in sarcoidosis has, however, not been described. Because acute sarcoidosis sometimes resembles malignant lymphoma, the possible rise of serum TK in sarcoidosis may be worthy of note.
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ranking = 0.00031172583601182
keywords = fever
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9/105. Immunoglobulin synthesis in vitro by cerebrospinal fluid cells in patients with meningoencephalitis of presumed viral origin.

    cerebrospinal fluid (CSF) cells from six patients with meningoencephalitis of presumed viral origin were incubated in the presence of labeled amino acids. The cells of two of the patients synthesized IgG, IgA, and IgM (one patient) in vitro. The CSF of these two patients had an elevated level of IgG with oligoclonal distribution. The newly synthesized IgG also had an oligoclonal distribution. CSF cells of the other four patients were not shown to synthesize immunoglobulins in vitro. The CSF of these patients had a normal level of IgG with polyclonal distribution. The results demonstrate that in some patients with virus meningoencephalitis an immunoglobulin synthesis takes place locally and that at least part of the IgG shows a restricted heterogeneity. The results also suggest the presence of stimulated lymphocytes in the CSF of the same patients.
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ranking = 1.0216569252531
keywords = virus, encephalitis
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10/105. Cytophagic histiocytic panniculitis with atypical lymphocytes in peripheral blood.

    A 26-year-old female patient developed high fever, hepatosplenomegaly and subcutaneous nodules. Atypical lymphocytes were present in the peripheral blood. skin biopsy revealed lobular panniculitis. bone marrow examination showed prominent phagocytosis by benign histiocytes. The diagnosis of cytophagic histiocytic panniculitis was made, and the disease has been well controlled with oral prednisolone. This is the first report of cytophagic histiocytic panniculitis with atypical lymphocytes in the peripheral blood, which are frequently seen in virus-associated hemophagocytic syndrome. This observation suggests that underlying viral infection may be one factor in the development of cytophagic histiocytic panniculitis.
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ranking = 1
keywords = virus, fever
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