Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A two-year-old girl with measles virus (MV) and chronic Epstein-Barr virus (EBV) infection developed lethal coronary aneurysmal arteritis accompanied by giant cell pneumonia, systemic lymphadenitis and hepatosplenomegaly. In her coronary arteries, lungs and aorta, cells containing intranuclear and intracytoplasmic inclusions, including syncytial giant cells, were detected, the presence of MV in the organs being proved by electron microscopic and immunofluorescent studies. Immunopathology further demonstrated MV to be disseminated in almost all organs other than lymph nodes. Clinical diagnosis of chronic EBV infection was established on the basis of persistent high titers of antibodies against capsid and early antigens of EBV and viral presence was confirmed by Southern blot hybridization in a mesenterial lymph node obtained at autopsy. To the best of our knowledge, this is the first description of MV association with coronary aneurysmal arteritis, raising the possibility that measles infection can cause severe vasculitis under immuno-suppressive states, such as that caused by chronic EBV infection.
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PMID:Measles associated with coronary arteritis. 196 61

Epstein-Barr virus (EBV) DNA was detected in polyclonal T cells that proliferated transiently in a 21-year-old male (referred to as H.J.) who underwent an apparently benign lymphocytosis (white blood cells, 31 x 10(6)/microL; lymphocyte, 79%) with fever, tonsillar swelling, lymphadenopathy, and hepatosplenomegaly. The symptoms and signs subsided mostly within a month of hospitalization. The major population of the lymphocytes at admission was positive for CD3, CD8 (4/8 ratio, 0.16), WT31, and DR antigen. Eight percent of the leukocytes were too blastoid to be classified as atypical lymphocytes of infectious mononucleosis (IM). The blastoid lymphocytes and the duration and degree of the lymphocytosis and hypergammaglobulinemia appeared inconsistent with IM, whereas the EBV serology indicated either EBV primary infection or a secondary alteration of normal seropositive EBV immunity. The genomic analysis of T-cell receptor beta chain in the peripheral blood mononuclear cells (PBMC) at admission with a C beta probe did not show a monoclonal rearrangement. EBV genome was detected in these cells, using the BamHI W and K probe, but not in the cells after discharge. Analysis of the EBV terminal repeat junctional sequence, using Xho I fragment of the latent membrane protein (LMP) probe binding with the terminus, did not show monoclonal or oligoclonal populations. EBV-associated nuclear antigen (EBNA) was detected in 36% of the PBMC at admission, but not in the later cells. These EBNA-positive cells were found to form rosette with sheep erythrocytes. The PBMC of six acute IM patients contained neither EBV DNA nor EBNA-positive cells. The observations in this case show a unique type of EBV infection in T cells that has not been previously reported.
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PMID:Detection of Epstein-Barr virus genome in benign polyclonal proliferative T cells of a young male patient. 216 93

We have had the opportunity to study a case of Chediak-Higashi syndrome (CHS) in the accelerated phase that was associated with Epstein-Barr virus (EBV) infection. The clinical course of a 12-year-old boy was characterized by fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia. However, in the terminal stage, the appearance of an atypical lymphoblastic leukocytosis was morphologically indistinguishable from acute lymphocytic leukemia, accompanied by benign histiocytosis with hemophagocytosis. Autopsy examination revealed an atypical lymphoid infiltration favoring EBV infection as the primary diagnosis. This case underscores the fatal consequences of EBV infection in CHS.
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PMID:Epstein-Barr virus infection in Chediak-Higashi syndrome mimicking acute lymphocytic leukemia. 216 46

Four patients from 4 to 24 years of age (3 males, 1 female) with generalized lymphadenopathy, hepatosplenomegaly, and intermittent fever associated with chronic active Epstein-Barr virus (EBV) infection were investigated. Laboratory data showed polyclonal gammopathy and a tendency for bone marrow suppression. Noteworthy were the extremely elevated immunoglobulin G (IgG) antibody titers to Epstein-Barr viral capsid antigen (VCA) (range, 10,240-81,920) and early antigen (EA) (range, 1,280-40,960). All patients had IgA antibodies to VCA and EA. Subtle, heterogeneous immune functional defects were observed in all four patients. Another unusual feature was our inability to establish spontaneous or B95-8 EBV-immortalized lymphoblastoid cell lines (LCLs) due to a marked cytopathic effect (CPE). Thus, we investigated for other viruses. Both IgG and IgM antibodies to adenovirus type-2 (Ad-2) were positive by enzyme-linked immunosorbent assay (ELISA) and immunofluorescence (IF) test, suggesting recent or activated Ad-2 infection had occurred. Dual active EBV and Ad-2 infections were likely etiologic in this severe chronic active EBV infection syndrome.
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PMID:Severe chronic active Epstein-Barr virus infection syndrome and adenovirus type-2 infection. 216 45

The results of clinical and Epstein-Barr virus (EBV) serological studies on nine Chediak-Higashi syndrome (CHS) patients are reported. Persistently elevated antibodies to the viral capsid antigen (VCA) and the restricted component of the early antigen complex (EA-R) developed in six patients who experienced primary EBV infection which either remained silent or were accompanied by clinical signs of infectious mononucleosis (IM). Hepatosplenomegaly and moderate lymphadenopathy, both clinical signs of the accelerated phase, remained detectable in the six patients for a long period of time after seroconversion. The clinical, serological, and histopathological observations are suggestive of a nonmalignant lymphoproliferative disease and consistent with an immunodeficiency to EBV. The abnormal serological responses to EBV in CHS are therefore considered manifestations of a chronic active EBV infection which may result in lethal lymphoproliferation. The three as yet seronegative CHS patients revealed no signs of the accelerated lymphoproliferative phase of the syndrome.
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PMID:Chronic active Epstein-Barr virus infection in patients with Chediak-Higashi syndrome. 301 35

A detailed clinicopathologic analysis of 52 cases of fatal infectious mononucleosis was performed. Fever, rash, generalized lymphadenopathy, hepatosplenomegaly, and blood cytopenias were the characteristic findings. Epstein-Barr virus infection was documented in 44 of the 52 patients. A triphasic process evolved in the blood and bone marrow of 43 patients. Early, the leukocyte count was elevated due to numerous atypical lymphoid cells, and the marrow was hyperplastic. Later, severe pancytopenia developed, and the marrow showed extensive infiltration by lymphoid cells with cellular necrosis and histiocytic hemophagocytosis. Terminally, the marrow showed massive necrosis with severe cellular depletion and marked histiocytic hemophagocytosis. The median survival time of the patients was six weeks. Opportunistic infections and/or acute hemorrhage were the major causes of death. We conclude that bone marrow damage secondary to an Epstein-Barr virus-associated hemophagocytic syndrome plays a major role in the death of patients with infectious mononucleosis.
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PMID:Fatal infectious mononucleosis and virus-associated hemophagocytic syndrome. 357 9

A 1-year-old infant girl had a chronic illness characterized by persistent intermittent fever, thrombocytopenia, granulocytopenia, and marked hepatosplenomegaly. Histological findings on liver biopsy 307 days after the onset of her illness disclosed marked mononuclear cell infiltration and moderate fibrosis in the portal and perilobular areas. Interferon-therapy induced no clinical and laboratory improvement, whereas the patient's clinical and biochemical findings responded to prednisone. Although these findings suggested a possible autoimmune mechanism, there were no significant findings in extensive immunological and serological tests consistent with autoimmune disease. The profile of antibodies to Epstein-Barr virus (EBV)-specific antigens in the patient demonstrated that this prolonged atypical illness was the result of persistent EBV infection.
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PMID:Chronic hepatitis associated with Epstein-Barr virus infection in an infant. 372 69

Two pediatric patients with life-threatening Epstein-Barr virus infections were studied immunologically and treated with acyclovir [9-(2-hydroxyethoxymethyl) guanine]. The patient with chronic active Epstein-Barr virus infection who experienced massive hepatosplenomegaly, pancytopenia, and failure to thrive demonstrated abnormalities of T and B lymphocytes. A second patient, with the X-linked lymphoproliferative syndrome, experienced a rapidly fatal course of acute Epstein-Barr virus infection which typifies this yet undefined immunodeficiency to Epstein-Barr virus. In each case, objective evidence for clinical improvement or antiviral effect of acyclovir treatment was not apparent. Abnormally productive Epstein-Barr virus infections did not appear to play a major role in the clinical syndromes observed. Current studies are focused on treatment of immunologically normal patients with early complicated Epstein-Barr virus infection.
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PMID:Treatment of life-threatening Epstein-Barr virus infection with acyclovir. 628 18

Severe chronic active Epstein-Barr virus (EBV) infection is a lymphoproliferative disease characterized by extremely high antibody titers to EBV, fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia, without any prior immunological abnormality. A spontaneous lymphoblastoid cell line was established from a 4-year-old boy with severe chronic active EBV infection. Immunofluorescence and Western blotting analyses showed that the cell line was of B cell origin and expressed Epstein-Barr nuclear antigens 1, 2 3a, 3b and 3c, and latent membrane protein 1, which are reported to be targets for EBV-specific cytotoxic T lymphocytes (CTL). The cytotoxicity of peripheral blood mononuclear cells derived from the patient and his HLA-identical sister was assayed against the cell line. The cell line was recognized and killed by anti-EBV CTL derived from the HLA-identical sister, but the patient's peripheral blood mononuclear cells had no cytotoxicity. We conclude that antigen presentation in the EBV-infected cells from the patient is intact and sufficient for generation of an EBV-specific CTL response. These observations suggest that severe chronic active EBV infection may not be caused by impaired EBV-antigen presentation of the infected cells but by impaired cellular immune responses to the virus. Our results also suggest the therapeutic possibility that this disease may be treated by adoptive transfer of EBV-specific CTL or bone marrow transplantation from an HLA-matched donor whose immune response to EBV is intact.
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PMID:Intact antigen presentation for Epstein-Barr virus (EBV)-specific CTL by a lymphoblastoid cell line established from a patient with severe chronic active EBV infection. 750 Sep 12

A 20-year-old female was admitted because of high fever, hepatosplenomegaly, severe hepatic dysfunction and coagulopathy. Peripheral blood showed pancytopenia and granular lymphocytes bearing the natural killer cell phenotype (CD2+CD3-CD16+CD56+CD57-TCR alpha beta-TCR gamma delta-) constituted 97% of leucocytes. Southern blot analysis of DNA obtained from peripheral blood mononuclear cells showed germ-line configuration of TCR beta, gamma and delta chain genes. EBV-DNA was detected in a single episomal form by using EBV-terminal repeat probe. Bone marrow findings were consistent with hemophagocytic syndrome and administration of VP-16 was effective transiently. After ten months she died from massive gastrointestinal bleeding. An in situ hybridization study identified EBV-RNA (EBER-1) in atypical lymphocytes infiltrating bone marrow, spleen and lymph nodes. Sections of liver showed steatosis and infiltration of T cells (CD3+ and EBER-1-negative) in the portal areas and few atypical lymphocytes in sinusoids. The patients developed an EBV-associated clonal proliferation of natural killer (NK) cells, but the clinical features were suggestive of chronic active EBV infection or virus-associated hemophagocytic syndrome (VAHS) rather than leukemia. Bone marrow transplantation for NK cell leukemia is an issue to be discussed.
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PMID:[Epstein-Barr virus associated natural killer cell leukemia: report of an autopsy case]. 778 57


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