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)

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

We report an unusual case of aggressive natural killer (NK)-like cytotoxic T-cell lymphoma in a previously healthy immunocompetent West African male. He presented with a fever of unknown origin, subsequently developed erythematous skin nodules, generalized lymphadenopathy, and hepatosplenomegaly, and then died of multiple organ failure. A skin nodule and lymph node biopsy showed an infiltrate of pleomorphic atypical medium and large lymphoid cells with extensive necrosis and prominent apoptosis. Peripheral blood and ascites also harbored these cells, with cytology revealing irregular nuclear folding and basophilic cytoplasm, and some with azurophilic cytoplasmic granules. Flow cytometry and immunohistochemistry demonstrated the expression of CD2, CD7, CD8, CD30, CD56, and cytoplasmic but not surface CD3. In situ hybridization demonstrated Epstein-Barr virus transcripts. A monoclonal T-cell receptor gamma chain gene rearrangement was detected by polymerase chain reaction. This is the first reported case of an NK-like T-cell lymphoma with these unusual features, making precise classification difficult. Some features suggest an NK1.1 or NKT lymphocyte origin. Because the earliest clinical manifestation was splenomegaly and abnormal liver function, the normal cellular counterpart may be a distinct subset of NK1.1 cells normally present in hepatosplenic sinusoids. This tumor disseminated early and pursued a fulminant clinical course, thus emphasizing the importance of early recognition and diagnosis.
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PMID:Aggressive Epstein-Barr virus-associated, CD8+, CD30+, CD56+, surface CD3-, natural killer (NK)-like cytotoxic T-cell lymphoma. 1175 78

A rare case of pyothorax-associated large B-cell lymphoma occurring in Hong Kong is reported. The patient was a 64-year-old Chinese male who presented with shortness of breath and pleuritic pain. Radiological examination revealed left pleural thickening associated with bilateral pleural effusion. Open biopsy of the thickened parietal pleura revealed occasional large malignant lymphoid cells of B lineage admixed with fibrin and hyalinised fibrous tissue. These lymphoma cells were shown to harbour both Epstein-Barr virus and human herpesvirus type 8 by in situ hybridisation and immunohistochemical study, respectively. There was no associated lymphadenopathy and hepatosplenomegaly. The clinicoradiological presentation and pathological findings thus fulfilled the criteria of the so-called pyothorax-associated large B-cell lymphoma. Awareness of this rare entity, together with diligent histological examination and proper application of ancillary investigative techniques, are essential for making a correct diagnosis. The co-infection with Epstein-Barr virus and human herpesvirus type 8 in this case also suggests a possible pathogenetic relationship between pyothorax-associated large B-cell lymphoma and primary effusion lymphoma.
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PMID:Pyothorax-associated large B-cell lymphoma: case report with emphasis on the potential diagnostic challenge. 1237 14

Diffuse nodular lymphoid hyperplasia (DNLH) of the intestine is an extremely rare lymphoproliferative disorder occasionally associated with non-Hodgkin lymphomas. We report an unusual case of DNLH of the entire colon, which resembled malignant lymphoma (particularly low-grade B-cell lymphoma) both on clinical and pathologic grounds. The patient was a 62-year-old Japanese woman who was found to have multiple polypoid lesions along the entire large intestine by colonoscopy. Abdominal computed tomography revealed hepatosplenomegaly and multiple mesenteric lymphadenopathy. Histologically, the lesion was characterized by numerous lymphoid follicles with active germinal centers and a diffuse infiltrate of lymphoid cells in the mucosa and submucosa. The present case appears to be an essentially benign condition bearing a resemblance, both clinically and histologically, to malignant lymphoma, and from which it can be distinguished by use of immunohistochemical or molecular analysis.
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PMID:Diffuse nodular lymphoid hyperplasia of the large bowel without hypogammaglobulinemia or malabsorption syndrome: a case report and literature review. 1249 Sep 83

Giant tumor cells and their varieties in the bone marrow were found in 7 patients with abnomal hematopoiesis phenomena. These cells were artificially devided into 5 kinds according to the difference of their morphology. Most of these cells were corresponding to lymphoid-monocytoid-macrophagocytoid cells with Wright's staining, cytochemical stainings, immunocytochemical stainings, flow cytometry examination, electron microscopy and pathologic study. The bone marrows were hypercellular and marked dysplastic hematopoiesis phenomena. Two of the 7 cases were diagnosed as malignant lymphoma with bone marrow biopsy. All cases characteristically showed no lymph node enlargement or hepatosplenomegaly or any local tumor mass. As to the prognosis of these cases, two patients died with survival time of 8 and 17 months, respectively, one was on critical condition at course of 10 months, and the other 4 cases were in comparatively stable condition with courses of 2.5 to 24 months. These patients seem to be a group of rare malignant lymphoid-monocytoid-macrophagocytoid proliferative diseases.
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PMID:[Giant Tumor Cells in the Bone Marrow and Their Significance] 1257 5

Hepatosplenic T-cell lymphoma is an uncommon neoplasm characterized by a lymphoid infiltrate within the sinusoids of the liver, spleen, and bone marrow, without significant nodal involvement. The majority of cases express the gammadelta T-cell receptor and are associated with an isochromosome 7q cytogenetic abnormality. Recently, a small number of cases have been reported that express the alphabeta T-cell receptor. Here, we report our findings of a case of an S100-positive hepatosplenic alphabeta T-cell lymphoma in a 20-year-old woman who presented with pancytopenia and hepatosplenomegaly. The case adds to the growing literature of hepatosplenic alphabeta T-cell lymphomas.
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PMID:Hepatosplenic alpha beta T-cell lymphoma: a report of an S100-positive case. 1265 97

Primary renal lymphoma is a rare disease. There is still no agreement on its development primarily in this non-lymphoid organ. Some of the reported cases are questionable because of the incomplete staging of extrarenal disease. In the present study, we describe a rare case of a 73-year-old white man presenting with right flank pain. Physical examination did not reveal any peripheral lymphadenopathy or hepatosplenomegaly. An abdominal computerized tomography (CT) showed a large tumor in the right kidney with no enlarged liver, spleen or lymph nodes. On morphological and immunohistochemical examination, the tumor fulfilled the criteria of anaplastic large cell lymphoma (ALCL). A review of the literature revealed that this is the first reported case of primary ALCL of the kidney.
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PMID:Primary anaplastic large cell lymphoma of the kidney. 1268 57

Myelofibrosis following peripheral T-cell lymphoma has rarely been reported. Described here is a case of peripheral T-cell lymphoma with myelofibrosis and elevated transforming growth factor beta (TGF-beta). A 69 years old male was admitted due to anemia and thrombocytopenia. His bone marrow showed fibrosis and was infiltrated with small lymphoid cells and a few residual normal hematopoietic cells. He had presented with hepatosplenomegaly and left inguinal lymph node swelling. Biopsy of the left inguinal lymph node revealed diffuse mature small lymphoid cells with atypical nuclei. Immunophenotyping of the small lymphoid cells were positive for CD3, CD8, TCR alphabeta and HLA-DR and were negative for CD4, CD19, CD20 and CD56. T-cell receptor beta-chain gene was rearranged in bone marrow cells. He was diagnosed as having peripheral T-cell lymphoma complicated with myelofibrosis. Chemotherapy was administrated which improved his pancytopenia and symptoms. Two years later, anemia and thrombocytopenia developed rather quickly, he died because of progression of myelofibrosis with severe pancytopenia.
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PMID:Peripheral T-cell lymphoma presenting with rapidly progressing myelofibrosis. 1268 59

We describe four classical cases of familial hemophagocytic lymphohistiocytosis (FHL), a macrophage-related, autosomal recessive fatal disorder. Parental consanguinity was present in three cases. All patients presented with fever, neurological involvement of varying degrees, hepatosplenomegaly, cytopenias, deranged liver function tests, and coagulogram, hypofibrinogenemia (three cases), and hyperlipidemia (one case). An antemortem diagnosis could not be made, although it was suspected in one case. Necropsy (done in three cases and postmortem liver biopsy in one case) revealed classical features of FHL. Florid lymphohistiocytic infiltrate exhibiting hemophagocytosis was seen in the bone marrow, liver, spleen, lymph nodes and brain (examined in two case). In addition to this, focal infiltrates were seen in the kidneys, lung, pancreas, testes, adrenals, and skin. Marked lymphoid depletion was seen in one case in the lymph nodes and spleen.
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PMID:Familial hemophagocytic lymphohistiocytosis: an autopsy study. 1274 74


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