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)

Human T-cell leukemia/lymphoma virus I can transform mature T-lymphocytes in vitro and is associated with the human T-cell cancer, adult T-cell leukemia/lymphoma. Adult T-cell leukemia/lymphoma is a distinct clinicopathological entity associated with leukemia, lymphadenopathy, hepatosplenomegaly, skin lesions, hypercalcemia, and lytic bone lesions. Although morphologically diverse it pursues an aggressive clinical course. Human T-cell leukemia/lymphoma virus III is associated with acquired immunodeficiency syndrome, which in its early stages shows follicular lymphoid hyperplasia; however, lymphoid atrophy is progressive and ultimately results in virtually total lymphoid depletion of lymph nodes. Patients with human T-cell leukemia/lymphoma virus III infections appear to have an increased risk of high-grade B-cell lymphomas and perhaps Hodgkin's disease.
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PMID:Lymph node pathology of HTLV and HTLV-associated neoplasms. 299 Jul 5

A patient with two attacks of glottis angioedema in a 15-day period without any apparent stimulus was studied. The complement profile of the patient revealed depletion of C4, C2, C1 inhibitor (C1INH) and C1q, with normal values of C3. Patient's offspring had a normal complement profile. Cytofluorographic analysis of the peripheral blood cells showed a marked increase of B cells. In the clotting study, a circulating lupus-like anticoagulant activity (LLA) was detected with a noticeable decrease of prothrombin time. Hepatosplenomegaly was confirmed by abdominal echography and CAT. From the liver biopsy it was concluded to be a lymphoproliferative process compatible with germinal center lymphoma. It is suggested that the neoplasm is probably the origin of the LLA and the cause of C1 activation, producing the biochemical defect of C1INH and the clinical symptoms of angioedema.
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PMID:Acquired C1-inhibitor deficiency associated with a lupus-like anticoagulant activity. 314 11

A 55-year-old man was admitted to our hospital with fever, ascites, generalized lymphadenopathy and hepatosplenomegaly. A cervical lymph node was biopsied and diagnosed as a diffuse mixed cell type B-cell malignant lymphoma with positive cytoplasmic IgM in plasmacytoid lymphocytes and immunoblasts. Serum protein electrophoresis disclosed a monoclonal peak and immuno-electrophoresis identified the abnormal protein as IgM kappa(k). Serum immunoquantitation revealed an IgM level of 1470 mg/dl. Bence-Jones protein of the k type was positive in the urine. Cryoglobulin with the characteristics of IgM was present in the serum. In peripheral blood, hemoglobin was 12.4 g/dl, WBC 26,500/microliters with increased abnormal cells and the platelet count 2.2 x 10(4)/microliters. Low fibrinogen and high FDP levels indicated the existence of disseminated intravascular coagulation (DIC). Gabexate mesilate (FOY) was administered at a dose of 1,000 mg/day for the DIC with very good response. After one course of combination chemotherapy (vincristine, cyclophosphamide, prednisolone, adriamycin), he achieved complete remission. However, three months later, he showed icterus and anorexia again with high levels of serum GOT and GPT and positive HBs antigen. On the 117th hospital day, he became abruptly developed right hemiplegia and coma. Cranial CT demonstrated massive thalamic bleeding in the left hemisphere with ventricular rupture, and he died on the same day.
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PMID:B-cell malignant lymphoma associated with monoclonal macroglobulinemia and cryoglobulinemia. 315 23

Medical records of 370 patients with sarcoidosis were reviewed. Of these, 32 had a computerized tomographic (CT) and/or ultrasound (US) examination of the abdomen. Two patients had extensive abdominal adenopathy: one was diagnosed by CT and the other by US. Both patients had conventional chest radiographic findings characteristic of sarcoidosis. In addition, five patients had hepatosplenomegaly; three had only hepatomegaly; three had only splenomegaly. There exists a small and previously unsuspected incidence of patients with extensive abdominal adenopathy in sarcoidosis. Although lymphoma and metastatic disease are far more common causes of extensive abdominal lymphadenopathy, sarcoidosis should be considered in the appropriate clinical setting. In many cases, correlation with conventional chest radiographs may be confirmatory.
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PMID:Abdominal lymphadenopathy in sarcoidosis. 329 92

Fourteen patients with the diagnosis of leukemia and one with lymphoma developed systemic candidiasis. Involvement of the liver (15 patients), spleen (nine patients), and kidney (five patients) was diagnosed by clinical, CT, and pathologic findings. The CT findings ranged from low-density lesions (11 livers, nine spleens, and five kidneys) to hepatomegaly or hepatosplenomegaly. All livers and three kidneys had positive biopsy findings for Candida. Two patients with diffuse splenic lesions underwent splenectomy and were proven to have candidiasis. During a 1 year follow-up, two patients developed hepatic calcifications and one developed renal calcifications. In proper clinical setting, CT should be done for simultaneous evaluation of the liver, spleen, and kidneys. These studies, when positive, are useful to guide percutaneous or open biopsy and to follow the results of therapy. However, regardless of the hepatic CT finding, biopsy should be obtained to establish the diagnosis and begin proper treatment.
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PMID:CT findings in hepatosplenic and renal candidiasis. 330 88

Morphologic and clinical features of 30 patients with peripheral T-cell lymphoma (PTCL) were studied with particular attention to bone marrow and blood manifestations. Twenty-four (80%) patients had marrow involvement with lymphoma in trephine biopsies at initial diagnosis; two other patients subsequently developed marrow involvement. The bone marrow lesions were diffuse in 58% of the cases and focal, nonparatrabecular in 42%. A morphologic spectrum of lymphoma cells was seen with cases classified into small cell, mixed cell, and large cell/immunoblastic lymphoma. The bone marrow lesions were characterized by a heteromorphous population of lymphocytes, prominent vascularity with endothelial cell proliferation, reticulin fibrosis, and a polycellular infiltrate composed of plasma cells, eosinophils, and histiocytes. The histopathologic features in bone marrow biopsies were not pathognomonic for PTCL; the differential diagnosis may include non-Hodgkin's lymphomas of B-cell type, polymorphous reactive lymphoid lesions, including those from patients with acquired immune deficiency syndrome (AIDS), angioimmunoblastic lymphadenopathy, Hodgkin's disease, and systemic mastocytosis. The patients ranged in age from 13 to 81 years (median, 61 years) and generally presented with constitutional symptoms, lymphadenopathy, and hepatosplenomegaly. Abnormalities in one or more hematologic parameters were common and, in general, related to the degree of bone marrow involvement. Hypocalcemia was found in 40% of the patients studied and hypercalcemia in 4%. The median survival for PTCL patients was 11 months. Patients with small cell lymphoma, large cell/immunoblastic lymphoma, and marked eosinophilia had the shortest median survivals.
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PMID:Bone marrow manifestations of peripheral T-cell lymphoma. A study of 30 cases. 349 Jan 73

Ten years after the onset of hydroa vacciniforme (HV), a 16-year-old boy developed edema and red induration of the face, ears, and dorsa of the hands. Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase of several serum enzymes. The vesicle was situated intraepidermally with thrombosis and hemorrhage underneath. This confirmed the diagnosis of HV. In addition, dense cell infiltrate was seen in deep dermis and subcutaneous tissue. Histologic and immunohistochemical studies of the dermal cell infiltrate and lymph node showed an infiltrate of helper T lymphocytes with an atypia and histiocytic cells (S100[-], alpha-subunit[+]). Hence, we concluded HV and malignant lymphoma coexisted in this patient.
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PMID:Coexistence of hydroa vacciniforme and malignant lymphoma. 349 Aug 32

One hundred forty-eight patients with newly diagnosed follicular lymphoma were treated over a 12-year period. Twenty-two patients received radiotherapy for stage I and II disease, followed by adjuvant chemotherapy in 14 patients. One hundred thirteen were treated at presentation with short courses of chemotherapy, most often with single-agent chlorambucil for bulky stage II and stages III and IV disease. Thirteen patients were managed expectantly until there was evidence of disease progression. The median survival was 9 years. Patients treated with radiotherapy for stage I and II disease had an 83% relapse-free survival, but those with bulky stage II or stages III and IV disease treated with chemotherapy pursued a remitting and relapsing course with a 70% response rate at initial and subsequent retreatments, but a median duration of remission of 4 years in stage III and 1 year in stage IV disease (P = .041). Patients were observed in relapse and retreatment was administered as appropriate, once every 33 months on average. Poor prognosis patients could be identified by a combination of the presentation characteristics: B symptoms, hepatosplenomegaly, anemia, and abnormal liver function. These factors predicted a poor response to treatment and correlated with a short survival. Histologic subgroups were not associated with differences in survival, but transformation to a diffuse high-grade lymphoma was observed in 23 of the 72 patients (32%) at risk, with a median follow-up of 6 years and 6 months, and was associated with a very poor prognosis. The present treatment strategy has proved successful for most patients with localized disease and those older patients with indolent small volume disseminated follicular lymphoma. New approaches are being investigated for the younger poor prognosis patients.
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PMID:Follicular lymphoma: prognostic factors for response and survival. 353 22

Primary lymphoma of the kidney is extremely rare; most lymphomatous renal masses represent extension from adjacent sites of disease or involvement by generalized disease (4,9,12). Three men and one woman, 45 to 71 years of age, presented with solitary renal masses clinically thought to be renal cell carcinoma. Each experienced abdominal pain, one with hematuria and one with "B" symptoms. Physical examination revealed no peripheral lymphadenopathy or hepatosplenomegaly. Lactic dehydrogenase (LDH) was elevated in three cases, and blood urea nitrogen (BUN) and creatinine were slightly increased in two. Two cases were diagnosed correctly from needle biopsy, with ultrastructural confirmation in one case and marker studies, DNA flow cytometry, and cytogenetics in the other. Because of a presumptive diagnosis of renal cell carcinoma, two patients underwent nephrectomy. Three cases were large-cell lymphoma, and one, small noncleaved cell lymphoma.
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PMID:Malignant lymphoma presenting as a renal mass: four cases. 355 31

A 66-year-old man with a 6-month history of sweating at night, generalized lymphadenopathy, hepatosplenomegaly, and paraproteinemia was diagnosed to have a Stage IV mantle zone lymphoma (MZL), which behaved aggressively. The neoplasm rapidly disseminated to extranodal sites--the skin, lungs, pleural cavity, and the central nervous system. The neoplasm did not respond to initial double-agent chemotherapy, but it did partially respond to multi-agent chemotherapy. In addition, the neoplasm had histopathologic features not reported previously in MZL--vascular invasion, massive extranodal infiltration, high mitotic count, and convoluted nuclei.
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PMID:High-grade mantle zone lymphoma. 367 7


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