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)

Reed-Sternberg (RS) and Hodgkin's (H) cells are considered to be the neoplastic cells in Hodgkin's disease (HD). Although most data suggest their lymphoid origin, the nature of these cells still remains a subject of controversy. Recently, a number of RS cells have been found to express an antigen that is also present on follicular dendritic cells (FDCs), asserting FDCs as the possible progenitor cells of H-RS cells. This prompted us to investigate whether these CD21-positive cases had distinct clinicopathologic characteristics. In a series of 94 examined cases of HD, we identified 9 CD21-positive ones (4 of 37 cases of nodular sclerosis, 1 of 41 mixed cellularity, and 4 of 12 lymphocyte depletion HD) without any other B-cell marker on paraffin sections. The patients varied in age from 16 to 82 years (median, 50 years) and included six men and three women. They had superficial or mesenteric lymphadenopathy without hepatosplenomegaly. Peripheral blood leukocytosis was seen in three patients. The clinical course was indolent, and all patients but one achieved an initial complete response with HD-based treatment regimens, although three of them relapsed. Morphologically, two subgroups could be delineated. Six of the cases were characterized, besides by the classic RS cells, by a varying number of the cells with the distinctive walnutlike or cerebrumlike nuclei and cytologically with cytoplasmic processes. Their fine structural examination also revealed villous processes, but no desmosomes. The other three cases had multinucleated RS cells often with triangular nuclei, but not cytoplasmic processes. The percentage of CD21-positive tumor cells ranged from less than 10% to 60% among the H-RS cells. These RS cells were positive for CD30 (9 of 9), CD15 (7 of 9), CD68 (1 of 8), fascin (8 of 8), S-100 protein (1 of 7), and epithelial membrane antigen (2 of 8) on paraffin sections. Notably, of eight cases examined on frozen sections, two showed immunostaining for DRC1, CD35, R4/23, and Ki-M4p. Only CD35 was also detected in the other two cases. Genotypic investigation showed germline configuration of the T-cell receptor beta and gamma chain genes and the immunoglobulin heavy chain gene in all eight cases examined. In situ hybridization showed Epstein-Barr virus sequences in four cases, three of which were examined by the terminal region analysis and showed the Epstein-Barr virus to be monoclonal. We concluded that in a small proportion (9.6%) of HD, H-RS cells might be derived from FDCs and that they appear to represent a distinct pathologic variant based on morphologic and phenotypic traits within the framework of HD.
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PMID:Hodgkin's disease expressing follicular dendritic cell marker CD21 without any other B-cell marker: a clinicopathologic study of nine cases. 1019 66

A 26-year-old man presented with a 38 degrees C fever of undetermined origin in November 1977. His fever subsided in 1 month, but recurred concomitantly with dysphagia on December 10. For this reason, the patient visited our out patient clinic on December 15, and was hospitalized because a mass was found radiographically in the left lower lung field. A contrast-enhanced computed tomographic scan of the chest revealed an irregularly-shaped mass encircling the esophagus. Based on open-lung biopsy findings, the mass was diagnosed as non-Hodgkin's lymphoma, diffuse pleomorphic type. Neither swelling of the superficial lymph nodes nor hepatosplenomegaly was detected, and a bone-marrow aspiration specimen disclosed no atypical cells. Ga scintigraphy found accumulation only in this region, suggesting the posterior mediastinum as the origin of the lymphoma. Reports of non-Hodgkin's primary lymphoma in the posterior mediastinum are rare. Further, to our knowledge, there have been no reports at all on the diffuse pleomorphic type to date. This case was accordingly considered to be of highly informative value.
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PMID:[Posterior mediastinal diffuse pleomorphic lymphoma]. 1021 48

We report a 74-year-old man who presented with a rash on the trunk showing clinical and histological features of non-Langerhans cell histiocytosis. Two years after presentation he developed weight loss, lymphadenopathy and hepatosplenomegaly; a diagnosis of lymphocyte-predominant Hodgkin's disease was made on lymph node biopsy. The cutaneous signs and lymphoma responded to chemotherapy. Taken in conjunction with previously published reports of associations between cutaneous histiocytoses and haematological malignancies, we recommend close observation of patients in whom a diagnosis of non-Langerhans cell histiocytosis is made.
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PMID:Non-Langerhans cell histiocytosis associated with lymphocyte-predominant Hodgkin's disease. 1056 22

The study aimed at the diagnosis of toxoplasmosis in 73 children with malignancy; 31 with lymphoma (22 with Hodgkin's and 9 with non-Hodgkin's lymphoma) and 42 with leukemia (34 with acute lymphoblastic leukemia and 8 with acute myelogenic leukemia). In positive cases toxoplasmosis was manifested by any of the following; fever, lymph node enlargement, neurological manifestations and/or hepatosplenomegaly. The indirect hemagglutination test (IHA) for toxoplasmosis detected 4 (5.4%) positive cases with malignancy, 2 with Hodgkin's lymphoma, one with non-Hodgkin's lymphoma and one with acute lymphoblastic leukemia. The immunoglobulin M enzyme-linked immunosorbent assay (IgM ELISA) detected only one (1.4%) case with Hodgkin's lymphoma. Immunoglobulin G (IgG) ELISA detected 6 (8.2%) positive cases, 3 with Hodgkin's lymphoma, one with non-Hodgkin's lymphoma and 2 cases with acute lymphoblastic leukemia. Polymerase chain reaction for detection of parasite DNA in blood (PCR) was the most useful in diagnosing toxoplasmosis with malignancy, as it was able to detect 9 (12.3%) positive cases; 5 (6.8%) with Hodgkin's lymphoma, one (1.4%) with non-Hodgkin's lymphoma and 3 (4.1%) with acute lymphoblastic leukemia. No positive toxoplasmosis cases were detected with acute myelogenic leukemia by any of the above methods.
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PMID:Diagnosis of toxoplasmosis in children with malignancy. 1094 13

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

We describe 4 cases of non-Hodkin's lymphomas that were interesting because of their curiosal clinical courses and spontaneous complete remissions during the course of high malignancy lymphoma. We present three of them for the first time in Poland. Case 1: a 61-year old woman was admitted to the hospital because of the headache, lasting for 4 months before hospitalization and right hemiparesis. CT scans revealed the presence of tumor in the temporo-occipital region. The diagnosis of B-cell lymphoma was established at histopathological examination of the postoperative material. Co60--therapy of these region was applied after the operation with good response. Case 2: a 38-year woman was admitted to the hospital because of L5-S1 spondylolisthesis to operate it. During the hospitalization haemolytic anaemia of unknown origin, thrombocytopoenia, splenomegaly, fever and rising acute insufficiency of kidneys, heart, liver and CNS were occurred. The patient died, despite applying corticosteroidotherapy. The diagnosis of intravascular lymphoma was established at postmortem examination. Case 3: a 51-year old woman was admitted to the hospital with diagnosis: anaplastic non-Hodgkin lymphoma B-cell type high malignancy established after the double histopathological examination of lymph nodes and biopsy of the lung. At the admission to the Department of Haematology we stated absolute regression of these changes. The patient had been only observed in the Outpatient Department over 1 year. She died after 6 months since the beginning of the relapse despite intensive chemotherapy. Case 4: a 43-year old man was admitted to the hospital because of great hyperleukocytosis, hepatosplenomegaly and neurological symptoms. The diagnosis: chronic prolymphocytic leukaemia was established. The cerebrospinal fluid examination showed presence of mononuclears which infiltrated CNS. CT scans of the brain revealed leucaemic infiltrations of the hemispheres and cerebellum. The patient died despite intensive therapy due to rising progressive multiorgan failure.
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PMID:[Unusual cases of non-Hodgkin's lymphomas--case reports]. 1123 49

The association of Hodgkin disease with monoclonal gammopathy has rarely been reported. We present a case of a 48-year-old woman with a history of autoimmune hemolytic anemia and Graves disease who presented with hepatosplenomegaly and a gamma-heavy-chain paraprotein. Histopathology of lymph node and bone marrow revealed nodular lymphocyte-predominance Hodgkin disease, while examination of the spleen revealed plasmacytosis consistent with gamma-heavy-chain disease. Following splenectomy, the patient has remained in complete remission for both conditions with no further treatment. To our knowledge, this is the first report of a patient with both gamma-heavy-chain disease and nodular lymphocyte-predominance Hodgkin disease. Given recent data indicating the B-cell nature of this form of Hodgkin disease, the authors propose that in this unique case there may be a clonal relationship between these 2 concurrent B-cell lymphoproliferative processes.
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PMID:Composite nodular lymphocyte-predominance Hodgkin disease and gamma-heavy-chain disease: a case report and review of the literature. 1137 Dec 36

An 8-years-old boy was admitted with fever of unknown origin, cervical lymphadenopathy and hepatosplenomegaly and weight loss. His mother's HIV infection was diagnosed two weeks before his hospitalization, so he was diagnosed as perinatally acquired AIDS. Serology and serial cultures were negative for viral infections, toxoplasmosis, chagas, tuberculosis and atypical mycobacterium. The patient met clinical and laboratory criteria for hemophagocytic syndrome (HS) that was confirmed on bone marrow aspirate and biopsy. A cervical lymph node biopsy was performed which was diagnosed as Hodgkin's disease (HD) diffuse fibrosis lymphocyte depletion subtype. EBERs in situ hybridization and LMP-1 immunohistochemistry on the lymph node biopsy established the EBV association. On the basis of a sequence of appearance of the clinical, laboratory and histological signs, HIV, EBV or HD may have triggered HS as the last fatal event in this pediatric patient.
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PMID:EBV-associated Hodgkin's disease in an HIV-infected child presenting with a hemophagocytic syndrome. 1169 13

A 30 year old man presented with late stage HIV disease and intrathoracic lymphadenopathy. Histology of a mediastinal biopsy suggested infective follicular hyperplasia or a peripheral T cell lymphoma. Subsequently, Epstein-Barr virus (EBV) infection was demonstrated in lymphocytes in the biopsy. Later, hepatosplenomegaly and peripheral lymphadenopathy developed. Histology of a cervical lymph node biopsy showed EBV associated diffuse large B cell (non-Hodgkin's) lymphoma.
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PMID:Progressive intrathoracic lymphadenopathy: EBV associated non-Hodgkin's lymphoma. 1187 51

We describe a case of B-prolymphocytic leukaemia (B-PLL) who, following a long-lasting remission with fluradabine, developed a Hodgkin's lymphoma (HL) with bone marrow involvement. A 75-yr-old male was found to have a lymphocytosis [white blood cell (WBC) count = 146 x 10(9) L(-1)], small volume axillary lymphadenopathy, and hepatosplenomegaly. The majority of circulating lymphocytes had a round nucleus and prominent single nucleolus. The patient did not respond to chlorambucil and then received fludarabine, achieving a good response lasting for 5 yr, when he manifested with B symptoms and pancytopenia. A diagnosis of HL in the bone marrow was made based on histology (Reed-Sternberg cells) and immunohistochemistry (CD30+, CD15+). Epstein-Barr virus (EBV) studies were negative. The patient was treated with chemotherapy but died 6 months later from disease progression.
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PMID:Prolymphocytic leukaemia and Hodgkin's lymphoma. 1240 13


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