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)

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

Four Venezuelan patients with the autosomal recessive Chediak-Higashi syndrome (CHS) were studied. The results confirm the severe reduction in natural killer (NK) cell activity, as previously described and showed also a decline in the activity of cells involved in antibody-dependent cellular cytotoxicity (ADCC). No defect was found in the production of immunoglobulins and of specific antibodies to measles, varicella, herpes simplex, and cytomegalo viruses. Two of the patients had extremely high antibody titers to the Epstein-Barr virus (EBV) specific viral capsid antigen (VCA), to the restricted (R) component of the EBV-induced early antigen complex, and to the EBV-associated nuclear antigen (EBNA). These two patients had enlarged livers, spleens, and lymph nodes indicative of the lymphoproliferative phase. The other two patients were initially negative for all EBV-associated antibodies but seroconverted subsequently and, in the course of a year, also developed high antibody titers to VCA and R. In one of these patients the primary infection was accompanied by moderate signs of infectious mononucleosis (IM) followed after more than 6 months by persistent hepatosplenomegaly. The other patient also developed signs of a lymphoproliferative syndrome with hepatosplenomegaly and jaundice and died 8 months later. Such high anti-R titers are seen frequently in Burkitt's lymphoma, but rarely in other conditions. It is likely that the high antibody titers reflect an increased production of VCA and R due to defective NK and ADCC cell activities so that productively infected B lymphocytes are no longer eliminated before they have synthesized maximal amounts of antigens. The high anti-EBNA titers suggest normal T lymphocyte function. The possibility that the accelerated, lymphoma-like phase of the CHS involves EBV-transformed cells is discussed.
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PMID:Elevated antibody titers to Epstein-Barr virus and low natural killer cell activity in patients with Chediak-Higashi syndrome. 630 71

Twenty five cases of Burkitt's lymphoma in Thai children were diagnosed at the Department of Pediatrics, Siriraj Hospital during the period of 13 years (January 1969 to April 1982). Males were more affected than females with the ratio of 1.7:1. The age ranged from 2 to 11 years with the median age of 4-5 years. The most common clinical manifestations were abdominal mass associated with nausea vomiting, abdominal pain, anorexia, weight loss and generalized lymphadenopathy which occurred in 50-60% of cases. Additional symptoms and signs included anemia, hepatosplenomegaly, edema and pleural effusion. Jaw tumor was found in only 37.5% of the patients. Definite diagnosis depended on the characteristic starry sky appearance of the lymph node biopsy or section of abdominal mass. In advance cases, the tumor cells could be discovered in bone marrow aspiration, ascitic fluid pleural fluid and cerebrospinal fluid. The typical blast cells were detected in the peripheral blood in 4 cases. Antibody to Epstein-Barr virus could be detected in almost all cases with high titers in some cases. Most patients responded very well to local irradiation and chemotherapy with prednisolone plus cyclophosphamide and vincristine or methotrexate. However, relapse occurred rapidly and 80% of the patients died within 3 months after diagnosis with the median survival of only 1 month. Five cases expired early before any specific treatment. The main causes of death were disease, sepsis, excessive bleeding and hyperkalemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Burkitt's lymphoma in Thai children: an analysis of 25 cases. 631 66

Malignant histiocytosis is characterized by systemic, progressive, and invasive proliferation of malignant histiocytes. The disorder is typically accompanied by fever, general fatigue, lymphadenopathy, and hepatosplenomegaly. A case of a 21-year-old woman with primary malignant histiocytosis of the oropharynx is reported. Histologic diagnosis from the biopsy specimen was confused by infiltration of normal-appearing histiocytes and inflammatory cells. The titers of Epstein-Barr virus and the Paul-Bunnell test were elevated without atypical lymphocytes. The patient died 3.5 months after the onset of symptoms. Autopsy revealed systemic neoplastic proliferation of malignant histiocytes. A review of literature on this subject revealed no cases of malignant histiocytosis primarily involving the oropharynx.
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PMID:Malignant histiocytosis in the oropharynx. 647 63

Angioimmunoblastic lymphadenopathy occurred in a 46-year-old man 16 months after an episode of infectious mononucleosis induced by Epstein-Barr (EB) virus. The features of infectious mononucleosis included fever, pharyngitis, lymph gland enlargement, hepatosplenomegaly, hyperbasophilic mononuclear cells, and IgM antibodies to EB virus, although heterophile antibodies were not detected. The illness was severe and prolonged and included an asymptomatic measles virus infection. Over a year later massive enlargement of the lymph nodes led to a biopsy, which showed a diffuse infiltration with lymphoid cells and a proliferation of arborising small vessels typical of angioimmunoblastic lymphadenopathy. In spite of corticosteroids, levamisole, chlorambucil, and radiotherapy, no remission occurred, and serious infections led to death 18 months after the onset. Viral infections with EB virus and measles virus associated with pre-existing or subsequent immunological changes probably resulted in the appearance of angioimmunoblastic lymphadenopathy.
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PMID:Angioimmunoblastic lymphadenopathy after infectious mononucleosis. 678 65

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

Mononucleosis is defined as atypical lymphocyte proliferation which causes clinical symptoms such as tonsillitis, lymphadenopathy, or hepatosplenomegaly. Mononucleosis syndrome is caused by cytomegalovirus (CMV), Toxoplasma, hepatitis virus, adenovirus, or other agents as well as by Epstein-Barr virus. The syndrome is immunologically characterized by the proliferation of activated T cells (HLA-DR+ T cells). We encountered three infants with hepatosplenomegaly who were diagnosed as primary CMV infection by the detection of anti-CMV IgM antibody. Although the patients were otherwise asymptomatic, analysis of lymphocyte subpopulations showed a decreased ratio of CD4+ to CD8+ T cells and augmented expression of HLA-DR antigen on T cells characteristic of infectious mononucleosis. We conclude that inapparent CMV disease may affect the immunologic status of infected children even if it is asymptomatic.
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PMID:Peripheral blood lymphocyte subpopulations in three infants with hepatosplenomegaly caused by cytomegalovirus infection. 764 91

A 23-year old woman was admitted to our hospital because of fever, leukocytopenia, thrombocytopenia and cervical lymphadenopathy. From pathological findings of the cervical lymphnode she was diagnosed as subacute necrotizing lymphadenitis (SNL). Bone marrow aspiration was hypocellular with proliferation of mature histiocytes with hemophagocytosis. Lactate dehydrogenase was elevated to 1863 IU/l and mild liver dysfunction was observed. Hepatosplenomegaly, lymphnode swelling in abdominal cavity, pleural effusion and pericardial effusion were detected. After glucocorticoid therapy all such disorders were normalized. In this course, Epstein-Barr (EB) virus genome was detected by a polymerase chain reaction method from peripheral mononuclear cells. After normalization, EB virus genome became not to be detected. From present case we concluded that EB virus was expanded in clinical course and was associated with pathogenesis of SNL.
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PMID:[Detected Epstein-Barr virus genome using polymerase chain reaction in peripheral mononuclear cells--subacute necrotizing lymphadenitis]. 769 34

A 19 year old man with a history of Crohn's disease treated with azathioprine and prednisone, died after a primary infection with Epstein-Barr virus. He had the characteristics of the virus associated haemophagocytic syndrome, a rare complication of viral infections, which consists of fever, constitutional symptoms, hepatosplenomegaly, liver function and coagulation abnormalities, and hypertriglyceridaemia. Additionally, there was pain, cytopenia, and histiocytic hyperplasia in the bone marrow, spleen, or lymph nodes. This severe complication has been reported previously in renal transplant patients, but not in those with inflammatory bowel disease taking azathioprine. The immunosuppressive therapy may have contributed to this fatal complication of infectious mononucleosis, and this complication should be considered when treating a patient with inflammatory bowel disease with azathioprine.
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PMID:Fatal infectious mononucleosis: a severe complication in the treatment of Crohn's disease with azathioprine. 788 36

The clinicopathologic features of 23 patients with hematophagic histiocytosis (HH) are described. All of them exhibited increased histiocytes associated with hemophagocytosis in the marrow. The patients usually presented with fever, hepatosplenomegaly, lymphadenopathy, and cytopenia. The underlying illnesses were heterogeneous, including non-Hodgkin's lymphoma in 17, systemic lupus erythematosus in one, diabetes mellitus in one, acute myelomonocytic leukemia in one, myelodysplastic syndrome in one, and unknown cause in two. Among 17 non-Hodgkin's lymphoma, 14 were peripheral T-cell lymphoma, two were B-cell lymphoma, and one was an undefined phenotype. Among 14 patients with peripheral T-cell lymphoma, six of the patients had nasal T-cell lymphoma. Five of these 14 patients initially diagnosed as malignant histiocytosis turned out to be T-lineage lymphoma after immunophenotypic studies. Active infections, most of viral origin, were documented in eight patients, including Epstein-Barr virus in three, cytomegalovirus in three, herpes simplex virus in three, Pseudomonas aeruginosa in one, Bacteroides vulgatus in one, and mycoplasma in one. Some of them had mixed virus and bacteria infection. Sixteen (70%) of our patients died of their acute illness within 10 weeks of the diagnosis of HH. In the past, the clinical and histologic differentiation between hematophagic histiocytosis and true histiocytic neoplasm (histiocytic medullary reticulosis/malignant histiocytosis) has proved difficult, but now these can be distinguished with immunohistologic, immunogenetic, and cytogenetic studies, especially in the cases of peripheral T-cell lymphoma with hemophagocytic syndrome.
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PMID:Hematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma. 792 83


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