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)

Reactive hemophagocytic syndrome, which is characterized by systemic proliferation of benign hemophagocytic histiocytes, usually presents as an acute febrile illness with pancytopenia and hepatosplenomegaly. The commoner diseases associated with the syndrome are infection and malignant lymphoma. In this report, eight cases of reactive hemophagocytic syndrome occurring in infants and young children are described. Unlike the disease occurring in adults, there is frequent occurrence of atypical mononuclear cells both in the peripheral blood and bone marrow. The morphological spectrum of these atypical cells is however still within that seen in infectious mononucleosis, and their reactive nature is substantiated by their spontaneous disappearance and subsequent recovery of the patients. It is important to distinguish this reactive proliferation from the neoplastic cells of 'malignant histiocytosis' or malignant lymphoma, since cytotoxic drugs are not warranted for treatment of this non-neoplastic condition.
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PMID:Reactive hemophagocytic syndrome in childhood--frequent occurrence of atypical mononuclear cells. 807 Jul 55

A 5 year old boy had a spiky fever accompanied by a mild pharyngitis, cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings revealed leukocytosis with 26% atypical lymphocytes, and liver dysfunction. A chest X-ray showed pneumonia and a considerable amount of pleural effusion. Serum antibody titers for cytomegalovirus (CMV) were elevated significantly and CMV-DNA (polymerase chain reaction) was detected in the pleural effusion. Only 13 cases of pleural effusion associated with infectious mononucleosis have been reported previously in the literature, but there was no documentation that proved CMV infection. The case reported here suggests that the pleural effusion was caused by the infiltration of mononuclear cells to the pleura as a result of systemic inflammation, and the possible alternative of host immune response against CMV was related to recent Varicella zoster virus infection.
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PMID:A case of cytomegalovirus mononucleosis associated with pleural effusion. 809 78

An 11-year-old girl presented with a typical serologically proven infectious mononucleosis with persistent fever, jaundice and hepatosplenomegaly in spite of steroid therapy. Laboratory tests showed pancytopenia, fibrinopenia and hypertriglyceridemia. The liver biopsy revealed an infiltration with hyperbasophilic cells. One month later, a slight improvement was noted and fever disappeared after 4 days on acyclovir therapy. The authors recall the spectrum of the macrophagic activation syndrome.
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PMID:[Epstein-Barr virus infection and syndrome of inappropriate macrophage activation]. 824 46

A case of a healthy 23-year-old woman is reported with cytomegalovirus mononucleosis as a result of infection of cytomegalovirus probably primary. The patient presented with symptoms of generalized adenopathy, migratory arthralgias and arthritis, hepatosplenomegaly, long lasting rash as well as complications of pneumonia and myocarditis. Because on histopathological examination of lymph node the Hodgkin-Reed-Sternberg-like cells were found a misdiagnosis of Hodgkin's disease was initially made. After about 8 weeks period there was a complete recovery. The current problems related to cytomegalovirus infection are presented.
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PMID:[Difficulties in diagnosis of cytomegalovirus mononucleosis syndrome]. 852 1

Thirty-one cases of infectious mononucleosis treated with Interfon-Alpha were reported. The dose of intramuscular injection was one million units per day for 5-7 days. The recovery course of fever, angina, lymphadenopathy and hepatosplenomegaly was much shorter in the study group than in the control group (27 cases). The results suggested that interfon-Alpha should be efficacious against EBV activity and might shorten the course of this disease.
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PMID:[Curative effect of Interfon-Alpha in children with infectious mononucleosis]. 920 28

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

The X-linked lymphoproliferative disease (XLP) is an inherited immunodeficiency characterized by an abnormal responses to infection with Epstein-Barr virus (EBV), resulting in fatal infectious mononucleosis, hypogammaglobulinemia, virus-associated hemophagocytic syndrome, and malignant lymphoma. Mutations in the gene coding for a T cell-specific SLAM-associated protein (SAP) have been recently identified in XLP patients. We report on a 1-year-old boy representing fulminant hemophagocytic syndrome. He developed high fever, lymphadenopathy, hepatosplenomegaly with liver dysfunction, and pancytopenia with marrow hemophagocytosis. EBV DNA was abnormally increased in the blood. Polymerase chain reaction failed to amplify SAP mRNA and genomic DNA products from the patient' As peripheral blood. A large deletion of the SAP gene was confirmed by fluorescence in situ hybridization (FISH). FISH analysis also disclosed that the patient's mother was a carrier. We conclude that FISH can be useful in the diagnosis of XLP with large deletions of the SAP gene and its carrier state.
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PMID:Large deletion of the X-linked lymphoproliferative disease gene detected by fluorescence in situ hybridization. 1081 94

This study describes the clinicopathologic features of 5 patients who developed a fulminant Epstein-Barr virus (EBV)-positive clonal T-cell lymphoproliferative disorder (LPD) after acute EBV infection. One additional patient developed a similar disorder in the setting of long-standing chronic active EBV infection. Detailed immunophenotyping, in situ hybridization for EBV early RNA-1 (EBER1) and polymerase chain reaction (PCR) analyses for immunoglobulin (Ig) heavy chain and T-cell receptor (TCR)-gamma gene rearrangements were performed on paraffin-embedded tissue from all patients. In addition, EBV strain typing and detection of the characteristic 30-bp deletion of the latent membrane protein-1 (LMP-1) gene were performed by PCR. Controls included 8 cases of uncomplicated infectious mononucleosis (IM). Patients included 4 males and 2 females with a median age of 18 years (2-37 years). Three patients were Mexican, 2 were white, and 1 was of Asian descent. All presented with fever, hepatosplenomegaly, and pancytopenia; 5 were previously healthy, but had a clinical history of a recent viral-like upper respiratory illness (1 week to 2 months), and 1 patient had documented chronic active EBV infection for 7 years. Serologic data for EBV were incomplete but titers were either negative or only modestly elevated in 3 cases. In 1 case serology was consistent with severe chronic active EBV infection. In the remaining 2 cases serologic studies were not performed. All patients died within 7 days to 8 months of presentation with T-cell LPD. On histologic examination, the liver and spleen showed prominent sinusoidal and portal lymphoid infiltrates of CD3(+), beta F1(+), EBER1(+) T cells lacking significant cytologic atypia. Two cases were CD4(+), 2 cases were CD8(+), and 2 cases had admixed CD4(+) and CD8(+) cells without clear subset predominance. All were TIA-1(+), CD56(-). Only rare B cells were noted. Marked erythrophagocytosis was present. Molecular analysis revealed identical T-cell clones in 2 or more sites (liver, spleen, lymph node) in 5 cases. All patients carried type A EBV; 4 cases had wild-type EBV-LMP, and 2 showed the 30-bp deletion. This fulminant T-cell LPD after acute/chronic EBV infection is characterized by hepatosplenomegaly, often without significant lymphadenopathy, fever, liver failure, pancytopenia, and erythrophagocytosis indicative of a hemophagocytic syndrome. EBV serology may be misleading, with lack of elevated titers. The presence of an EBER1(+) T-cell infiltrate with scant B cells should alert one to this diagnosis. Although cytologic atypia is minimal, studies for T-cell clonality confirm the diagnosis. (Blood. 2000;96:443-451)
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PMID:Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome. 1088 4

The presence of Epstein-Barr virus (EBV) in the Hodgkin's/Reed-Sternberg (HRS) cells of a significant proportion of cases of Hodgkin's lymphoma (HL) is a matter of consideration when a case of presumptive HL has to be differentiated from infectious mononucleosis (IM). A 15-y-old boy was admitted with a presumptive diagnosis of extranodal HL, based on the biopsy of a painless ulcer on the right mandibular alveolar crest. Histologic examination of the lesion was consistent with mixed cellularity HL. The patient additionally presented with hepatosplenomegaly and regional lymphadenopathy. Serology for EBV was indicative of acute infection. Histological examination of regional lymphoid tissue was consistent with immunologic activation due to primary EBV infection. The patient was left untreated, under close observation. All clinical findings resolved within 3 mo and EBV viral capsid antigen (VCA) IgM antibodies converted to negative after 6 mo. A 3-y follow-up period was uneventful.
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PMID:Reed-Sternberg cells in atypical primary EBV infection. 1123 57

Epstein-Barr virus-associated hemophagocytic syndrome (EBV-AHS) is often associated with fatal infectious mononucleosis. However, the animal model for EBV-AHS has not been developed. We reported the first animal model for EBV-AHS using rabbits infected with EBV-related herpesvirus of baboon (HVP). Eleven of 13 (85%) rabbits inoculated intravenously with HVP-producing cells developed fatal lymphoproliferative disorders (LPD) between 22 and 105 days after inoculation. LPD was also accompanied by hemophagocytic syndrome (HPS) in nine of these 11 rabbits. The peroral spray of cell-free HVP induced the virus infection with increased anti-EBV-viral capsid antigen-IgG titers in three of five rabbits, and two of these three infected rabbits died of LPD with HPS. Autopsy revealed hepatosplenomegaly and swollen lymph nodes. Atypical lymphoid T cells expressing EBV-encoded small RNA-1 infiltrated diffusely in many organs, frequently involving the lymph nodes, spleen, and liver. Hemophagocytic histiocytosis was observed in the lymph nodes, spleen, bone marrow, and thymus. HVP-DNA was detected in the tissues and peripheral blood from the infected rabbits by polymerase chain reaction or Southern blot analysis. Reverse transcriptase-polymerase chain reaction revealed both HVP-EBNA1 and HVP-EBNA2 transcripts, suggesting latency type III infection. These data indicate that the high rate of rabbit LPD with HPS induction is caused by HVP. This system is useful for studying the pathogenesis, prevention, and treatment of human EBV-AHS.
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PMID:An animal model for human EBV-associated hemophagocytic syndrome: herpesvirus papio frequently induces fatal lymphoproliferative disorders with hemophagocytic syndrome in rabbits. 1129 May 71


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