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)

One case of T-cell lymphoma with atypical malignant cells is reported. Some of the clinical features, morphological characteristics and functional activity (erythrophagocytosis) of malignant cells suggested malignant histiocytosis. The malignant disease started with splenomegaly and developed with hepatomegaly, bone marrow infiltration, discrete lymphadenopathy and leukaemic picture. Proliferated cells were characterized by ambiguity. In addition to phagocytic capability, presence of complement receptors and ultrastructural features proper to the macrophagic lineage, the cells expressed T-cell determinants (E receptors, T3, T4 and T11 antigens) and were peroxidase and esterase-negative. Erythrocytes were partially or completely dehaemoglobinized and presented the phenomenon of autolysis in different stages of development. Because this lymphoma is difficult to diagnose and apparently resistant to therapy, its recognition and further study are warranted.
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PMID:A case of erythrophagocytic T4 lymphoma. Immunological and morphological peculiarities. 169 69

A young man with bleeding symptoms, mild thrombocytopenia and abundant marrow megakaryocytes was classified as having idiopathic thrombocytopenic purpura. Neither prednisone therapy nor splenectomy modified the clinical picture. Subsequently, a severe defect of platelet aggregation and release reaction was demonstrated. Fifteen months after the onset of bleeding symptoms, fever and hepatomegaly appeared and the diagnosis of T cell non Hodgkin lymphoma was made on the basis of a histologic review of paraffin sections of the spleen. Chemotherapy induced remission of the lymphoma, disappearance of bleeding symptoms and normalization of the platelet count and function.
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PMID:Qualitative and quantitative platelet defect with bleeding symptoms as presenting feature of non Hodgkin lymphomas. 172 Jul 56

We describe the clinical and laboratory findings of 78 adult patients with T-prolymphocytic leukemia (T-PLL) studied over the last 12 years. The main disease features were splenomegaly (73%), lymphadenopathy (53%), hepatomegaly (40%), skin lesions (27%), and a high leukocyte count (greater than 100 x 10(9)/L in 75%) with nucleolated prolymphocytes. A variant form with small, less typical cells was recognized in 19%. Membrane markers defined a postthymic phenotype TdT-, CD2+, CD3+, CD5+, CD7+; in 65%, the cells were CD4+ CD8-, in 21%, they coexpressed CD4 and CD8, and, in 13%, they were CD4- CD8+. Serology for human T-cell leukemia/lymphoma virus Type-I (HTLV-I) was negative in the 27 cases investigated. Cytogenetic analysis in 30 cases showed a consistent abnormality of chromosome 14, usually inv (14), with breakpoints at q11 and q32 in 76% of cases. Trisomy 8, including iso8q, was shown in 53%; t (11;14)(q13;q32) was documented in one case; and one had a normal karyotype. The clinical course was progressive with a median survival of 7.5 months. Thirty-one patients were treated with 2' deoxycoformycin and 15 responded (3 complete remissions and 12 partial remissions); the response rate (48%) increased to 58% in patients with a CD4+ CD8- phenotype. The median survival of responders was 16 months and of nonresponders 10 months; other treatments were less effective. T-PLL is a distinct clinico-pathologic entity with aggressive course and characteristic chromosome abnormalities. A subgroup of patients may benefit from deoxycoformycin.
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PMID:Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. 174 86

Twenty five consecutive patients with fever of unknown origin (FUO) who underwent diagnostic laparatomy (DL) are reviewed. There were 14 females and 11 males, with a mean age of 34 years. The main symptoms and signs besides fever were malaise, weight loss, varied abdominal complaints, peripheral lymph nodes enlargement, hepatomegaly and splenomegaly. The main laboratory abnormalities were: anemia, leukocytosis, and mild alterations in liver function tests. CT scan was performed in 14 patients: hepatomegaly, splenomegaly and or retroperitoneal nodes were found in 10 of them. During laparotomy, an extensive exploration of intra-abdominal organs was performed, taking multiple biopsies for histopathologic and microbiologic analysis. Splenectomy was performed in 17 patients, prophylactic appendectomy in four, and cholecistectomy in one. Laparotomy was useful to establish a diagnosis in 64 percent of cases. The most frequently diagnosed pathologies were lymphoma and tuberculosis. Postoperative morbidity was 12% and mortality was 4%. Mean follow-up was 29 months. When preoperative data were analyzed, no predictive factors were found for a laparotomy with diagnostic success. It is concluded that DL is a useful last-step procedure in the diagnostic work-up for patients with FUO.
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PMID:Diagnostic laparotomy in fever of unknown origin. 186 93

Herein we describe the isolation, physicochemical characterization and preclinical evaluation of a water-soluble biologic response modifier extracted from Sclerotium glucanicum. Alkaline extraction of insoluble S. glucanicum exopolymers produced a soluble scleroglucan composed of a triple-helical beta-1,3-linked glucopyranose backbone with single beta-1,6-linked glucopyranosyl branches every third subunit. Scleroglucan has a weight average molecular mass of 1.56 x 10(6) Da, a weight average root mean square distance from the center of gravity of the molecule to its farthest elements of 51.8 nm, a polydispersity (weight-average molecular mass/number average molecular mass) of 1.83 and intrinsic viscosity of 3.081 dl/g. Scleroglucan (250 mg/kg, intravenously) stimulated in vivo murine macrophage phagocytic activity (66%, P less than .001) and increased in vitro macrophage tumor cytotoxicity against syngeneic tumor targets by 124% (P less than .05). Scleroglucan enhanced (P less than .001) murine bone marrow proliferation in a biphasic manner by up to 328%. Scleroglucan therapy increased survival of mice challenged with syngeneic lymphoma, melanoma or adenocarcinoma. AKR/J mice bearing syngeneic lymphoma (1 x 10(3) cells, intraperitoneally) demonstrated increased (P less than .001) long-term survival (100% vs. 0%, greater than 64 days). C57Bl/6J mice bearing syngeneic melanoma B16 (5 x 10(5) cells, subcutaneously) demonstrated increased long-term survival (64% vs. 0%, P less than .05). C57Bl/6J mice bearing syngeneic adenocarcinoma BW10232 (1 x 10(5) cells, subcutaneously) demonstrated increased (P less than .05) median survival time. In addition, scleroglucan prophylaxis increased resistance of mice to challenge with Staphylococcus aureus, Candida albicans and mouse hepatitis virus A-59. Scleroglucan did not induce toxicity or hepatomegaly. We conclude that: 1) a branched, water-soluble beta-1,3-linked scleroglucan biologic response modifier can be extracted from S. glucanicum; 2) scleroglucan will stimulate immunity, modify experimental neoplastic disease and increase resistance to microbial challenge; and 3) scleroglucan shows promise as an immunopotentiating drug.
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PMID:Isolation, physicochemical characterization and preclinical efficacy evaluation of soluble scleroglucan. 190 59

We report the diagnostic, prognostic and therapeutic features of non-Hodgkin's lymphoma in eight patients in whom the disease was seen as a primary tumor of the liver. This series illustrates the variety of situations in which lymphoma might be diagnosed: (a) abdominal pain and hepatomegaly (three cases), (b) incidental finding at evaluation of a patient with cirrhosis (two cases), (c) secondary neoplasm after treatment for Hodgkin's disease (one case) and (d) complication of AIDS (two cases). In most cases, clinical and/or radiological features were nonspecific. However, the combination of the following features must be considered as suggestive: occurrence of an apparently primary hepatic tumor in an immunocompromised patient, absence of the usual serum tumor markers and increased serum lactic dehydrogenase activity. The final diagnosis was based on histological examination of specimens obtained by ultrasonically guided liver biopsies or at surgery. All cases belonged to unfavorable histological subtypes. Immunohistochemical findings on paraffin-embedded sections demonstrated the B-lymphocyte lineage of the seven tumors available for study. In the three patients without coexisting disease, complete remission was obtained by surgery alone or combined with chemotherapy. In the two patients with coexisting cirrhosis, outcome was rapidly unfavorable, with death occurring less than 3 mo after diagnosis. Among the three immunocompromised patients, two experienced a rapid unfavorable outcome, and the remaining one was in complete remission after surgery and chemotherapy. In conclusion, primary non-Hodgkin's lymphoma of the liver arising in patients without coexisting disease has a slow progression and might be successfully treated by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Non-Hodgkin's lymphoma presenting as a primary tumor of the liver: presentation, diagnosis and outcome in eight patients. 202 91

In order to determine the extent of liver abnormalities occurring during acquired immunodeficiency syndrome, the available histological analyses of liver samples (32 biopsies, 52 autopsies) from 71 AIDS patients, for the period 1982-1986, were studied retrospectively. Hepatomegaly was the most common clinical symptom (23 patients, 32.4%), while jaundice was rare, being seen in only 5 cases (7%). Progressive anicteric cholestasis was the most frequently observed biological anomaly (29/52, 55.7%). Ten patients had liver infections: 2 Mycobacterium tuberculosis, 8 Mycobacterium avium intracellulare. Cytomegalovirus was present in 3 patients and 1 individual was infected with Cryptococcus neoformans. Granulomatous hepatitis was associated with these infectious agents in 11 patients, but remained unexplained in 11 others. Three patients had cholangitis (2 with CMV inclusions, 1 unexplained). Among the 32 biopsies, 5 elucidated the origin of unexplained fever. Kaposi's sarcoma of the liver was found in 10/52 autopsy samples (19%) and hepatic lymphoma in 2 cases. Non-specific histological lesions were common: inflammation of the portal spaces (48 cases, 67.6%), steatosis (32 patients, 45%), peliosis hepatis (9 cases, 12.6%) and sinusoidal dilations (39 cases, 54.9%).
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PMID:[Hepatic involvement in AIDS. A retrospective clinical study in 71 patients]. 217 55

We present two cases of primary malignant lymphoma of the liver, a very unusual localization of primary malignant lymphoma since only 31 similar cases were reported in the literature. The main symptoms included: hepatomegaly, fever, slight cholestasis and non specific alteration of the liver appearance on ultrasonography or computed tomodensitometry examination. Diagnosis was established in both cases by histopathological demonstration of infiltration by large lymphoid cells. Immunophenotyping in one case confirmed a B cell phenotype. Primary malignant lymphomas of the liver are high-grade malignant large-cell tumours and the immunophenotype, when known, is of the B type. The prognosis of these tumours is not well known due to their extreme rarity. They appear to be highly chemosensitive and cure may be obtained in the long-term in some cases. These data justify careful histological assessment in all cases of hepatomegaly of a tumoral nature.
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PMID:Primary malignant lymphoma of the liver. Report of two cases and review of the literature. 219 36

The association and presentation of malignant disease of the liver with fulminant hepatic failure has been described sporadically, but the absence of a large series has meant that malignancy is rarely considered in the differential diagnosis of such hepatic failure. We describe three cases and review the best documented reports in the literature. Review of 25 patients shows that in most cases, excluding lymphoma, the liver is massively replaced by tumor that often spreads in an intrasinusoidal pattern. The diffuse nature of malignant spread results in a relative failure of diagnostic imaging and thus the diagnosis is frequently made after death, which occurs a mean 7.8 days after hospital admission. Review of the clinical features and investigations in these cases shows that, when hepatomegaly and ascites are present in middle-aged or older patients at the time of admission, malignancy should be considered as the cause. Why the liver should be massively replaced in such patients remains obscure.
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PMID:Primary and secondary malignant disease of the liver and fulminant hepatic failure. 220 55

Retrospective review of 291 solid tumor and lymphoma patients undergoing autologous bone marrow transplantation (BMT) was performed to determine the influence of pretransplant characteristics and preparative regimen to the development of hepatic venoocclusive disease (VOD). Twelve patients (4.1%) developed a clinical syndrome of right upper quadrant (RUQ) tenderness or hepatomegaly, jaundice, and ascites, with or without encephalopathy, within 40 days of marrow reinfusion. Evidence of metastatic liver disease was the only pretransplant characteristic predictive for VOD (P = .0002). Sex, age, histology, hepatitis B serology, and elevated liver function tests were not predictive. No individual preparative agent had a significant effect on the development of VOD. However, a single 2-hour infusion of carmustine (BCNU) (greater than or equal to 450 mg/m2) led to an increased incidence of VOD when compared with the same dose administered in a fractionated schedule (P = .0258) when given with two other chemotherapeutic agents. Seven of eight autopsy specimens confirmed the clinical diagnosis of VOD. The four patients in whom clinical VOD resolved had lower median peak bilirubins (7.3 v 15.9 mg/dL), lower median peak creatinines (2.1 v 4.1 mg/dL), and relatively quick engraftment of neutrophils (mean, 18.7 days). One of the four patients in whom VOD resolved had other grade 4 (life-threatening) toxicities in contrast to eight of eight who succumbed. In summary, VOD is an uncommon complication in autotransplantation of solid tumors and lymphomas. Our data suggest caution in selecting patients with known metastatic liver disease and consideration of a fractionated BCNU schedule especially in combination with other alkylating agents.
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PMID:Hepatic venoocclusive disease in autologous bone marrow transplantation of solid tumors and lymphomas. 221 5


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