Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A simian homologue of Kaposi's sarcoma-associated herpesvirus (KSHV), the eighth human herpesvirus (HHV8), was isolated from a simian immunodeficiency virus (SIV)-infected rhesus macaque (Macaca mulatta) that developed a multicentric lymphoproliferative disorder (LPD). This simian rhadinovirus is genetically similar to a recently described rhesus rhadinovirus (RRV) (Desrosiers, R.C., V.G. Sasseville, S.C. Czajak, X. Zhang, K.G. Mansfield, A. Kaur, R.P. Johnson, A.A. Lackner, and J.U. Jung. 1997. J. Virol. 71:9764-9769) and is designated RRV 17577. RRV 17577 was experimentally inoculated into rhesus macaques with and without SIV(mac239) infection to determine if RRV played a role in development of the LPD observed in the index case. In contrast to control animals inoculated with SIV(mac239) or RRV alone, two animals coinfected with SIV(mac239) and RRV 17577 developed hyperplastic LPD resembling the multicentric plasma cell variant of Castleman's disease, characterized by persistent angiofollicular lymphadenopathy, hepatomegaly, splenomegaly, and hypergammaglobulinemia. Hypergammaglobulinemia was associated with severe immune-mediated hemolytic anemia in one RRV/SIV-infected macaque. Both RRV/SIV-infected macaques exhibited persistent RRV viremia with little or no RRV-specific antibody response. The macaques inoculated with RRV alone displayed transient viremia followed by a vigorous anti-RRV antibody response and lacked evidence of LPD in peripheral blood and lymph nodes. Infectious RRV and RRV DNA were present in hyperplastic lymphoid tissues of the RRV/SIV-infected macaques, suggesting that lymphoid hyperplasia is associated with the high levels of replication. Thus, experimental RRV 17577 infection of SIV-infected rhesus macaques induces some of the hyperplastic B cell LPDs manifested in AIDS patients coinfected with KSHV.
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PMID:Induction of B cell hyperplasia in simian immunodeficiency virus-infected rhesus macaques with the simian homologue of Kaposi's sarcoma-associated herpesvirus. 1049 21

AIDS is frequently expressed through gastrointestinal o abdominal symptoms. In addition, patients with AIDS or ARC frequently have hepatic and biliary symptoms, while pancreatic alterations are found in 4-30% of patients hospitalised for AIDS. Since AIDS patients are immunodepressed, they are subject to opportunistic infection often multifocal and the pathological processes can be present simultaneously. About 2/3 of patients have enlarged liver, steatosis, splenomegaly, lymphoadenopathy, cholecystic and biliary tract abnormalities, alterations of liver function tests, and abdominal discomfort in the upper right quadrant. Jaundice is rare and hepatic failure is not common. Hepatic biopsy is often necessary to establish the diagnosis. The hepatic localisation of an opportunistic pathogenic agent is generally a sign of systemic dissemination which is expressed as granulomatous hepatitis (atypical mycobacteria, frequently mycobacterium avium, or M. tuberculosis representing the reactivation of latent diseases), peliosis hepatis, infection from CMV, HSV, EBV, Pneumocystis carinii, and mycotic infections. Coinfections with the hepatic virus (HBV, HDV, HCV) are also often present. Pharmacological damage may also be present (mainly caused by antibiotic therapies). Neoplasia are rare (hepatic Kaposi's sarcoma associated with cutaneous and gastrointestinal manifestations, or generally metastatic lymphoma). Damage of the biliary tract usually develops after other manifestations of the illness; the most frequent pictures are cholestatic syndromes and cholangitis, while cholecystitis and jaundice are rare. Pancreatic lesions are generally asymptomatic. They are diagnosed during autopsy and are caused principally by opportunistic agents.
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PMID:[Hepatic and pancreatic disease in patients with acquired immunodeficiency syndrome (AIDS)]. 1051 57

Multicentric Castleman's disease (MCD) is a lymphoproliferative disorder that can be defined based upon both clinical and pathological characteristics. The clinical features of this frequently fatal disease include fever, generalized lymphadenopathy, fatigue, splenomegaly, hepatomegaly, and pancytopenia. Recently, severe forms of this disease have been diagnosed in HIV positive patients. Human herpesvirus type 8 (HHV-8) DNA sequences have been detected in peripheral blood mononuclear cells (PBMCs) of patients with Kaposi's sarcoma and MCD, regardless of HIV infection status. Treatment and outcomes in HIV associated MCD are generally unfavorable. We recently treated two HIV-positive patients diagnosed with aggressive MCD with daily oral etoposide (50 mg). The first patient had relapsed on several occasions despite previous therapy with doxil, paclitaxel, and oral ganciclovir. The second patient was treatment naive. Both patients had HHV-8 detectable by polymerase chain reaction in PBMCs, widespread tumor, and B-type symptoms when therapy was initiated. In both cases remissions (documented by computerized tomography) have been durable, 1.5 and 6 months, respectively, with minimal side effects. Oral etoposide may be a safe, tolerable, and active agent in MCD.
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PMID:Treatment of HIV-associated multicentric Castleman's disease with oral etoposide. 1142 Dec 97

Castleman disease is considered a reactive lymphadenopathic picture with two clinical forms: one localized, frequent in immunocompetent patients and another multicenter one that is more characteristic in immunodepressed patients. Two cases of Castleman disease multicenter in HIV positive patients with Kaposi's sarcoma are presented. Both patients have multiple adenopathies, hepatomegaly and symptoms B on diagnosis. One of them had a favorable response to chemotherapy treatment and another died. A review of the concept of multicenter Castleman disease and its pathogenic relationship to human herpes virus 8 (HHV-8) is done.
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PMID:[Multicenter Castleman disease in AIDS. Its relationship with HHV-8 or herpes virus associated to Kaposi's sarcoma. Study of two cases]. 1652 82

Castleman's disease is a group of rare lymphoproliferative disorders. The plasmablastic multicentric Castleman's disease is frequently discovered in HIV-infected individuals in association with Kaposi sarcoma (HHV-8). Thirty-five year old male presented to our care with the main compliant of severe back pain for one week. His past medical problems include acquired immune deficiency syndrome diagnosed 12 years prior and Kaposi sarcoma, currently on highly active antiretroviral therapy (HAART). Radiographic imaging revealed hepatomegaly and diffuse lymphadenopathy. The HIV viral load was <20 polymerase chain reaction copies/mL, absolute CD4 count was 453 cells/mcL (490-1740 cells/mcL) and CD8 count was 4142 cells/mcL (180-1170 cells/ mcL). Excisional biopsy of the left supraclavicular lymph node was performed with pathological findings of HHV8+ Kaposi sarcoma in the background of multicentric Castleman's disease (plasmacytic variant). No evidence of transformation into large B-cell or plasmablastic lymphoma was noted. He was discharged on HAART and follow up to receive chemotherapy with cyclophosphamide, adriamycin, vincristine plus prednisone was started and rituximab plus prophylaxis for pneumocystis carinii. Multicentric Castleman's disease has become more relevant in recent years due to its association with HIV and HHV-8 (Kaposi sarcoma) and its potential to progress into plasmablastic B-cell lymphoma. The progression of MCD to B-cell lymphoma is a concern, especially in patients with HIV infection because it precludes the worst outcome and a high mortality, despite treatment. The most intriguing part of this case is that MCD occurred in a HIV-positive on HAART. This case signals a warning that a high suspicion for MCD can be justified even in those HIV-positive patients on HAART because the possibly of progression to plasmablastic B-cell lymphoma.
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PMID:Multicentric Castleman's Disease and Kaposi's Sarcoma in a HIV-Positive Patient on Highly Active Antiretroviral Therapy. 2527 27


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