Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.7.7.48 (transcriptase)
 9,479 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

In the summer of 2001, Usutu virus (USUV) was isolated for the first time in Europe, from an episode of mass mortality in Eurasian blackbirds (Turdus merula). In the present study, 40 of the birds (representing three species), confirmed as cases of USUV infection, were examined by four methods (histopathology, immunohistochemistry [IHC], in-situ hybridization [ISH] and reverse-transcriptase polymerase chain reaction [RT-PCR]). The major macroscopical finding was hepatosplenomegaly; histologically, neuronal necrosis, myocardial lesions, and coagulative necrosis of the liver and spleen were observed. IHC with cross-reactive polyclonal antibodies to West Nile virus detected viral antigen predominantly in brain neurons (40/40 birds; 100%), myocardial fibres (25/32; 78%), cells of the splenic capsule (29/33; 88%), renal glomeruli (22/35; 63%), tunica muscularis of intestines (17/22; 77%), proventricular glands (16/19; 84%), lungs (18/33; 55%) and hepatic Kupffer cells (7/38; 18%). ISH with an USUV-specific oligonucleotide probe demonstrated viral nucleic acid predominantly in brain neurons (40/40; 100%), myocardial fibres (24/33; 73%), splenic macrophages (12/34; 35%), renal tubular cells (19/36; 53%), tunica muscularis of intestines (13/32; 41%), proventricular glands (19/22; 86%), lungs (7/34; 21%) and hepatic Kupffer cells (12/38; 32%). All of 33 birds tested additionally by USUV-specific RT-PCR gave positive results.
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PMID:Pathology and viral distribution in fatal Usutu virus infections of birds from the 2001 and 2002 outbreaks in Austria. 1527 57

A 14-year-old child presented with generalized lymphadenopathy and massive hepatosplenomegaly. Peripheral smear and bone marrow examination were suggestive of Ph' positive chronic myeloid leukemia (CML) in chronic phase. However, lymph node biopsy showed extramedullary blast crisis with evidence of myeloid and T cell markers in blasts. Reverse transcriptase-polymerase chain reaction from lymph node aspirate revealed transcript for bcr-abl p210. Thus, we present here a unique case of childhood CML with extramedullary biphenotypic blast crisis (myeloid/T cell type) at initial presentation with bone marrow remaining in chronic phase. This case provides further evidence to the highly heterogeneous presentation of CML.
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PMID:Biphenotypic extramedullary blast crisis as a presenting manifestation of Philadelphia chromosome-positive CML in a child. 1745 89

It is extremely rare that a patient with anaplastic large cell lymphoma (ALCL) demonstrates circulating lymphoma cells. A 10-year-old Japanese boy was presented with high-grade fever and cough. The physical examination revealed marked hepatosplenomegaly with ascites and lymphadenopathy in the cervical and periauricular areas. The white cell count was 26.2x10(9)/L with 95% of abnormal lymphoid cells, which were small to medium-sized with a high nucleus/cytoplasm ratio, basophilic cytoplasm, condensed nuclear chromatins, and 1 or 2 distinct nucleoli, hemoglobin 6.4 g/dL, and platelet 0.9x10(9)/L. A flow cytometric analysis of abnormal cells in both the peripheral blood and bone marrow samples was strongly positive for CD30 on their cell membranes. Karyogram and fluorescent in situ hybridization showed abnormal cells to have a characteristic chromosomal translocation, t(2;5)(p23;q35). Reverse transcriptase-polymerase chain reaction of peripheral blood cell-derived mRNA also indicated the fusion gene product of anaplastic lymphoma kinase and nucleophosmin. Subsequently, the patient was diagnosed to have ALCL with a rare clinical feature of a peripheral leukemic presentation, and his disease revealed to be refractory to chemotherapy. On the basis of the 11 childhood cases of ALCL with leukemic presentation so far published and reviewed herein, the prognosis is very poor.
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PMID:Anaplastic large cell lymphoma in leukemic presentation: a case report and a review of the literature. 1877 64