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)

Twenty-six cases of histologically proven malignant histiocytosis are presented. Patient ages ranged from 20 months to 82 years, and a 10:3 male preponderance was seen. Clinical presentation is similar to that of malignant lymphoma and leukemia; histologic differentiation is required. The frequency of intrathoracic involvement at the time of presentation is similar to that of non-Hodgkin's lymphoma. Retroperitoneal lymph node invasion was readily seen on five of the eight lymphograms performed. Hepatosplenomegaly was a common finding, and three of the patients had evidence of bone involvement during the course of their illness. While there are no radiographic findings specific to malignant histiocytosis, the presence of hepatomegaly should suggest this possibility. The establishment of the correct diagnosis is important to guide choices in chemotherapy.
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PMID:Radiographic manifestations of malignant histiocytosis. 97 May 32

A 75-year-old man was admitted to our hospital because of hepatosplenomegaly, generalized lymphadenopathy and lymphocytosis in February, 1989. The leukocyte counts were 93,200/microliters with 95% small lymphocytes which expressed surface membrane immunoglobulin (SmIg) M, D and kappa. Histological finding of the cervical lymph node was diffuse small cell lymphoma. A diagnosis of chronic lymphocytic leukemia (CLL) was made. He was followed up without chemotherapy. In January, 1990, he was re-admitted because of progressively enlarged lymph nodes and increased white blood cell counts, up to 183,200/microliters with 98% lymphocytes. He was treated with vincristine, cyclophosphamide, prednisolone. The leukocyte counts decreased to 5,000/microliters and lymph node swelling decreased in size. In April, 1990, generalized lymphadenopathy re-appeared. The biopsied lymph node specimen showed diffuse large cell non-Hodgkin lymphoma (NHL-DL). The lymph node cells were found to express SmIgM and kappa. The diagnosis of Richter's syndrome was made. DNA analysis using Southern blot method revealed identical immunoglobulin heavy and kappa chain gene rearrangements in the two neoplasms. These findings suggest that the CLL cells and the NHL-DL cells originate from the same clone in this case.
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PMID:[Richter's syndrome with identical immunoglobulin gene rearrangements]. 190 17

Primary pancreatic lymphoma is extremely rare. A case of the primary pancreatic lymphoma was described. A 74-year-old man was admitted to our hospital on May 26, 1990, because of epigastralgia and 5-kg weight loss. On admission, physical examination revealed mild epigastric tenderness without hepatosplenomegaly and superficial lymphadenopathy. The serum amylase level and trypsin level were normal but serum CA19-9 was slightly elevated. The diagnosis of the pancreatic cancer (body and tail) was strongly suspected on subsequent examination by US, CT, ERP and angiography. Partial pancreatectomy and splenectomy were performed. Pathological examination of the tumor in the pancreas showed diffuse non-Hodgkin's lymphoma and B-cell origin. Peripancreatic fat was involved but peripancreatic lymph nodes and spleen were intact, which strongly suggested that the isolated lymphoma was pancreatic origin.
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PMID:[A case of the primary pancreatic lymphoma]. 206 88

Eight elderly women with primary non-Hodgkin's lymphoma were treated with a combination of surgery, chemotherapy and radiation with excellent outcome. Clinically, the patients presented with thyromegaly and did not have lymphadenopathy or hepatosplenomegaly. On histological examination, the lymphomas resembled diffuse follicle-centre cell tumours. All patients showed extension beyond the thyroid capsule and lympho-epithelial lesions; 3 displayed kappa-light-chain restriction and 2 lambda-monoclonality. Thyroid lymphomas fall into the spectrum of mucosa-associated lymphomas, which have a better prognosis than their equivalent nodal-based counterparts.
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PMID:Primary thyroid lymphomas. A clinicopathological study of 8 cases. 223 90

To elucidate the clinicopathologic features of non-Hodgkin's lymphoma (NHL) in Taiwan, 123 adult patients with proven NHL were studied. They were classified according to the international working formulation as: low grade (LG), 12.2%; intermediate grade (IG), 42.3%; and high grade (HG) lymphoma, 45.5%. The most common subtypes were diffuse large cell (26.8%) and large cell immunoblastic (26.8%) lymphomas. Follicular lymphoma accounted for only 8.9% (11 cases). Complete remission rates for LG, IG and HG lymphomas were 53%, 35% and 34%, respectively. LG lymphoma had a significantly better survival than that of IG and HG lymphomas. The IG lymphoma encompassed a heterogeneous group of patients with varying prognoses but the overall survival curve was indistinguishable from that of HG lymphoma. Clinically, 66% of HG, 77% of IG and 86% of LG lymphoma presented with advanced disease. LG lymphoma had high frequencies of hepatosplenomegaly (30-50%) and bone marrow involvement (53%), whereas skin, bone and central nervous system involvement occurred exclusively in IG and HG lymphomas. Mild anemia was common and occurred in 40-50% of the patients. Hyperimmunoglobulinemia was found in 50-60% of all 3 grades of lymphoma, monoclonal gammopathy in 3 cases of IG lymphoma, and hypercalcemia in 4 cases of IG and HG lymphomas. Elevated serum lactate dehydrogenase occurred mainly in IG and HG lymphomas and was an important prognostic factor. In conclusion, the characteristic features of NHL in Taiwan include: (1) a high proportion of HG lymphoma and low proportions of LG and follicular lymphomas; (2) a heterogeneous patient composition of IG lymphoma with an unfavorable overall prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Adult non-Hodgkin's lymphoma in Taiwan area: a clinicopathologic study of 123 cases based on working formulation classification. 276 10

The early phase of the disease in 30 children referred to this Clinic with the diagnosis of rheumatic disease and whose condition was ultimately diagnosed as malignancy was analysed. A correct diagnosis was established after a period of 1-22 months. thus acute leukemia, non-Hodgkin's lymphoma, Hodgkin's disease and solid tumours were diagnosed in 13, 7, 3, 7 consecutive patients. It was demonstrated that in an early stage of neoplastic diseases, signs may occur which are typical also of rheumatic diseases. Fever, arthralgia or arthritis, hepatosplenomegaly, lymphadenopathy and signs of cardiac involvement predominated in the early picture of the disease. The need for diagnostic investigations ruling out neoplastic diseases has been emphasized. They should be performed even in children fulfilling the diagnostic criteria for rheumatic diseases. Attention has been called to the importance of carefully performed hematological and morphological analysis.
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PMID:Differential diagnosis of neoplastic and rheumatic diseases in children. 348 89

Skeletal involvement in patients with non-Hodgkin's lymphoma (NHL) is common, although direct involvement of the joints is unusual. We describe 2 adults who presented with features suggestive of a diagnosis of rheumatoid arthritis, but who were found to have diffuse NHL of the synovium. Results of a review of the literature, and assessment of the few similar cases in which NHL presented in the joint, suggest that the lymphoma may mimic either a monarticular or polyarticular synovitis, without lymphadenopathy or hepatosplenomegaly. Radiographic demonstration of associated bone destruction is the best evidence for non-Hodgkin's lymphomatous arthropathy in patients with rheumatic symptoms.
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PMID:Non-Hodgkin's lymphoma of the synovium simulating rheumatoid arthritis. 382 57

Serum lactate dehydrogenase (LDH) activity is increased in many tumor-bearing patients and can be used as a prognostic marker. We studied serum LDH concentration in 94 consecutive patients with non-Hodgkin's lymphoma who were histologically classified according to the Kiel Classification and were grouped according to the Non-Hodgkin's Lymphoma Pathologic Classification Project Working Formulation. 74 patients were studied at diagnosis, and 20 of them (27%) had an LDH level higher than 250 U/l. High LDH levels were more frequent in cases of true histiocytic, high-grade, and intermediate-grade malignancy lymphoma (4 of 7, 7 of 14, and 7 of 20, respectively) than in cases of low-grade lymphoma (2 of 33). A close relationship of LDH to several prognosis-related disease features was found, including general symptoms, bulky disease, big mediastinal tumor, huge hepatosplenomegaly, bone marrow involvement, and a leukemic syndrome. LDH was higher than normal in a high proportion of cases who were studied in relapse (13 of 20, 65%). These data suggest that in non-Hodgkin's lymphomas the LDH serum concentration is not independent of other disease features, so that the prognostic value of LDH is probably lower than expected from previous studies. Serum LDH activity decreased to normal in all cases of complete remission, but also in cases of partial remission, suggesting that measuring enzyme activity is of a limited usefulness for detecting and monitoring minimal residual disease. For that purpose, LDH isoenzyme studies would be more appropriate.
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PMID:Serum LDH concentration in non-Hodgkin's lymphomas. Relationship to histologic type, tumor mass, and presentation features. 643 91

A 36-year-old man with adult T-cell leukemia gradually improved to the point of complete remission without any anticancer treatment. The patient was referred to our hospital because of the appearance of morphologically abnormal lymphocytes. Thirty percent of the peripheral blood white cells were morphologically abnormal lymphocytes which had lobulated or indented nuclei, and were E rosette-forming and mature T-cell antigen-positive. No hepatosplenomegaly, lymphadenopathy or mediastinal mass was noted. He had a cough with sputum one and a half years before entry. After admission, skin nodules occurred and were diagnosed histologically as non-Hodgkin's lymphoma, diffuse pleomorphic. Serum antibodies against an adult T-cell leukemia-associated antigen were detected at a titer of 1:40. Chest X-ray films, a pulmonary function test, bronchographic examination, bronchoalveolar lavage and transbronchial lung biopsy demonstrated no relationship between the pulmonary symptoms and the adult T-cell leukemia. After treatment with antibiotics and a beclomethasone inhaler, the symptoms decreased, and then the abnormal lymphocytes and skin nodules disappeared. The spontaneous, complete remission has continued for four years.
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PMID:Spontaneous, complete remission in adult T-cell leukemia: a case report. 660 31

Simian T-cell leukemia virus type 1 (STLV-1), a type C retrovirus associated with leukemia/lymphoma in Old World monkeys, is closely related to human T-cell leukemia virus type 1, the etiologic agent of adult T-cell leukemia/lymphoma in humans. In a colony of 3200 baboons, the prevalence of antibodies to STLV-1 is more than 40%. Seropositivity is more frequent in female baboons than in males and increases with age. Of 27 STLV-1 antibody-positive baboons with non-Hodgkin's lymphoma, 20 were females and 7 were males, ranging in age from 3 to 21 years (mean, 13 years). Non-Hodgkin's lymphoma was not found in STLV-1 antibody-negative baboons. Clinical signs and laboratory findings were variable but generally included lethargy, low body weights, anemia, dyspnea, lymphadenopathy, hepatosplenomegaly, pneumonia, nodular skin lesions, and leukemia with or without multilobulated lymphocytes in peripheral blood. Radiography revealed pulmonary infiltrates consistent with pneumonia in 17 of the baboons. Serum chemical values were normal except for hypercalcemia in one baboon. Lymphocytosis was found in 18 of the baboons, with leukemia diagnosed in 11. At necropsy, variable enlargement of lymph nodes and other lymphopoietic tissue was usually found. Pale tan to white space-occupying foci typical of proliferative lymphoid tissue were often found in various organs, including lungs, spleens, livers, skin, and hearts. The lungs in 14 baboons had thickened pleuras, congestion,edema, and large tan to brown areas of consolidation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Spontaneously generated non-Hodgkin's lymphoma in twenty-seven simian T-cell leukemia virus type 1 antibody-positive baboons (Papio species). 790 50


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