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)

Primary lymphoma of the uterine corpus (PLUC) is an extremely rare neoplasm. We report a case of PLUC in a 78-year-old woman with vaginal bleeding without hepatosplenomegaly, adenopathies, or bone marrow infiltration, classified as stage I. A diagnosis of diffuse large B-cell lymphoma was made in endometrial curettage tissue. Immunohistochemical study showed tumoral cells of B-cell nature. Two different polymerase chain reaction (PCR) techniques showed immunoglobulin heavy chain gene rearrangement and we could not demonstrate, with PCR technique, either Epstein-Barr virus or papilloma virus infection. Total hysterectomy with bilateral salpingo-oophorectomy was carried out and adjuvant chemotherapy was given. She was alive and free of disease after a follow-up period of 7 years, and the patient has been in perfect health.
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PMID:Large B-cell lymphoma of the uterine corpus: case report with immunohistochemical and molecular study. 919 Sep 90

Virus associated hemophagocytic syndrome (VAHS) are a heterogeneous group of disorders in which viral infection is associated with a proliferation of hemophagocytic histiocytes through the reticuloendothelial system. We report the case of a 21-year-old Korean man who presented to us with high fever, marked hepatosplenomegaly, severe hepatic dysfunction, coagulopathy, pancytopenia and marked panhypogammaglobulinemia. Bone marrow aspiration and biopsy showed histiocytes proliferation with active phagocytosis of red cells and neutrophils. Primary Epstein-Barr (EB) viral infection at presentation was confirmed by the presence of IgM antibody to viral capsid antigen (VCA) with absence of antibody to EB viral nuclear antigen (EBNA). A liver biopsy performed one month after the presentation showed erythrophagocytic histiocytes within the sinusoids. EB virus was demonstrated in the liver biopsy tissue by DNA PCR method, and EBER mRNA in situ hybridization.
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PMID:Epstein Barr virus associated hemophagocytic syndrome--a case report. 936 4

Renal involvement is rare in chronic active Epstein-Barr (EB) virus infection. We report a case of a 7-year-old girl with recurrent EB virus infection. She had fever, lymphadenopathy, hepatosplenomegaly, and persistently high titres of IgG to EB virus capsid antigen (VCA) and IgG to EB early antigen with low titres of IgM to VCA. She showed mild haematuria and proteinuria, but had no symptoms of renal failure. Renal biopsy revealed immune complex-mediated glomerulonephritis, which may have been due to a persistently high titre of antibody against EB virus. In addition, a peculiar form of tubulointerstitial nephritis was found. The morphology was characterized by a papillary infolding of the tubular epithelial cell layer into the tubular lumen. The interstitium was surrounded by the infolded epithelium and contained a large number of B-cell dominant lymphocytes. EBV-encoded RNA 1 (EBER-1) gene was detected in the nuclei of some tubuloepithelial cells by in situ hybridization and may have been associated with the pathogenesis of tubulointerstitial nephritis.
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PMID:Epstein-Barr virus genome-positive tubulointerstitial nephritis associated with immune complex-mediated glomerulonephritis in chronic active EB virus infection. 967

Bone marrow hemophagocytosis may occur as an incidental finding, or it may be a manifestation of a systemic and potentially lethal disorder. When systemic, the proliferation is termed hemophagocytic lymphohistiocytosis (HLH), a clinicopathologic entity characterized by a widespread proliferation of benign hemophagocytic histiocytes, fever, pancytopenia, deranged liver function, and frequently coagulopathy and hepatosplenomegaly. A variety of infectious agents, including Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpesvirus 6 (HHV6), and parvovirus B19 (PVB19), have been associated with HLH, but the relative frequency of each using one technique has not been evaluated. In addition, infectious causes of incidental bone marrow hemophagocytosis, not occurring in the setting of HLH, have not been evaluated. Review of bone marrow reports from bone marrow examinations done between December 1986 and June 1997 showed that 20 children aged 2 months to 15 years had bone marrow examinations that indicated hemophagocytosis. Archival materials from 19 patients were successfully retrieved, and DNA was extracted from archived unstained coverslips with subsequent polymerase chain reaction for EBV, CMV, HHV6, and PVB19 genomic DNA. DNA extracted from 16 bone marrow specimens of age-matched children was used as negative controls. Eleven of the 19 patients fulfilled the clinical and pathological criteria for HLH; the remaining eight patients had isolated hemophagocytosis without a systemic presentation. Viral DNA was detected in 8 of 11 patients with HLH but in none of eight patients with isolated hemophagocytosis. EBV was present in five of the bone marrows, followed in frequency by HHV6, CMV, and PVB19. Infection with more than one agent was present in three patients. Only one control patient was positive for HHV6 DNA; the remaining control patients were negative for all viruses. Viral infection, detected by PCR analysis of bone marrow, is a common finding in patients with HLH but not in patients with isolated bone marrow hemophagocytosis. This technique may provide another marker to aid in the diagnosis of HLH and suggests a different cause of hemophagocytosis occurring in patients with and without HLH.
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PMID:Polymerase chain reaction amplification of archival material for Epstein-Barr virus, cytomegalovirus, human herpesvirus 6, and parvovirus B19 in children with bone marrow hemophagocytosis. 978 44

A 44-year-old woman with a history of intermittent fever for several years was admitted because of burn on her leg. On admission, she had hepatosplenomegaly and fever. Antibiotic therapy was started for bacterial infection of the burn. She lost her appetite and IVH was started. During the treatment, high fever appeared and chest X-ray films showed multiple nodular infiltrates throughout both lung fields. Candida albicans was isolated from IVH catheter culture and pulmonary candidiasis was suspected. Her fever and lung involvements were successfully treated with fluconazole. During the course, serum anti-EB-VCA-IgG antibody persisted at a high titer and anti-EBNA antibody remained negative. EB virus DNA was detected in the peripheral blood and bone marrow. Thus, she was diagnosed as chronic active EB virus infection.
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PMID:[A case with chronic active EB virus infection accompanied with pulmonary candidiasis]. 991 18

A 28-year-old man was admitted to our department with intermittent fever, hepatosplenomegaly and pancytopenia. Liver parameters and serum ferritin were markedly elevated. Bone marrow biopsy showed hypocellularity, histiocytic hyperplasia, and hemophagocytosis consistent with a virus-associated hemophagocytic syndrome (VAHS). There was serological evidence of chronic active hepatitis B and acute hepatitis C virus infection. The patient died despite aggressive immunosuppressive and supportive treatment. Autopsy revealed signs of acute viral hepatitis with cholestasis. Histiocytes engaged in hemophagocytosis were observed in bone marrow and spleen. The condition was interpreted as VAHS associated with chronic active hepatitis B and acute hepatitis C virus infection. To our knowledge this is the first report of a hemophagocytic syndrome in that setting.
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PMID:Fatal virus-associated hemophagocytic syndrome associated with coexistent chronic active hepatitis B and acute hepatitis C virus infection. 1036 94

We describe a case of peripheral T-cell lymphoma, unspecified (REAL) with co-existence of follicular dendritic cell (FDC) proliferation and EB virus-infection. A 55-year-old Japanese man complained of generalized lymphadenopathy and physical examination showed systemic lymphadenopathy, and hepatosplenomegaly. HTLV-1 antibody titers and gamma-globulin level were within normal limits. Histopathologic examination of the right cervical lymph node showed peripheral T-cell lymphoma, unspecified (REAL classification). A diffuse infiltration of lymphoma cells with abundant small venules were found throughout the lymph node. There were few arborizing venules. An irregular meshwork of FDCs was found by immunostaining with DRC-1 and CD21 in the area rich in venules, but not in the area of diffuse lymphoma cell-infiltration. EBER-1 RNA-in situ hybridization showed positive signal on the nuclei of mainly non-neoplastic B immunoblasts. The present case, therefore, was regarded as a rare case of peripheral T-cell lymphoma, unspecified with FDC proliferation and EB virus infection.
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PMID:Peripheral T-cell lymphoma other than angioimmunoblastic T-cell lymphoma (AILD), with follicular dendritic cells proliferation and infection of B immunoblasts with Epstein Barr virus. 1074 55

Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and prompt therapy have the potential to reduce mortality. Leukemias, lymphomas and central nervous system tumors account for more than one half of new cancer cases in children. Early in the disease, leukemia may cause nonspecific symptoms similar to those of a viral infection. Leukemia should be suspected if persistent vague symptoms are accompanied by evidence of abnormal bleeding, bone pain, lymphadenopathy or hepatosplenomegaly. The presenting symptoms of a brain tumor may include elevated intracranial pressure, nerve abnormalities and seizures. A spinal tumor often presents with signs and symptoms of spinal cord compression. In children, lymphoma may present as one or more painless masses, often in the neck, accompanied by signs and symptoms resulting from local compression, as well as signs and symptoms of systemic disturbances, such as fever and weight loss. A neuroblastoma may arise from sympathetic nervous tissue anywhere in the body, but this tumor most often develops in the abdomen. The presentation depends on the local effects of the solid tumor and any metastases. An abdominal mass in a child may also be due to Wilms' tumor. This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. A tumor of the musculoskeletal system is often first detected when trauma appears to cause pain and dysfunction out of proportion to the injury. Primary care physicians should be alert for possible presenting signs and symptoms of childhood malignancy, particularly in patients with Down syndrome or other congenital and familial conditions associated with an increased risk of cancer.
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PMID:Recognition of common childhood malignancies. 1077 55

A 26-year-old female was admitted because of multiple fractures in lower extremities. While in the hospital, she developed a high fever and generalized skin eruption. Physical examination revealed bilateral cervical lymphadenopathy and mild hepatosplenomegaly. The white cell count was 11,200 with 11% atypical lymphocytes. Serum GOT, GPT, LDH were markedly elevated. Infectious mononucleosis was suspected, but the serological test for EB virus did not show evidence of acute EB virus infection. Anti-HSV, CMV, hepatitis A virus antibody titers also did not show significant change during the coarse. The serological test for HHV-6 only showed increased titer of IgM and IgG antibodies. Rapidly elevated IgG antibody titer was indicative of reactivation of HHV-6. So, she was diagnosed as mononucleosis-like syndrome caused by HHV-6, probably reactivated infection. Her symptoms gradually disappeared during a month.
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PMID:[A case with infectious mononucleosis-like syndrome caused by human herpes virus-6 infection]. 1078 82

Infection-associated hemophagocytic syndrome (IAHS) is a form of the reactive hemophagocytic syndrome. IAHS is associated with viral, bacterial, fungal, mycobacterial, rickettsial and protozoal infections and with various malignant neoplasms. A more accurate designation for this acquired form of the syndrome is reactive hemophagocytic syndrome (HS). Reactive HS is characterized by malaise, fever, hepatosplenomegaly, lymphadenopathy, cytopenia, hypertriglyceridemia, hypofibrinogenemia and hemophagocytosis. Cyclosporin A, VP-16, high-dose steroids, and intravenous immunoglobulin (IVIG) have been used in the treatment of IAHS. Here, a 10-year-old girl with reactive HS due to possible viral infection was treated successfully with cyclosporin A and IVIG. Fever disappeared on the third day, complete blood count reached normal levels on the sixth day and hepatosplenomegaly disappeared on the ninth day after treatment. We believe cyclosporin A and IVIG may be used in the treatment of reactive HS, at least in selected patients. Further studies are required to confirm its role as first-line therapy for children with IAHS.
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PMID:Successful treatment of reactive hemophagocytic syndrome with cyclosporin A and intravenous immunoglobulin. 1093 88


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