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)

We analysed the clinical and pathologic features of 42 patients with immunologically confirmed peripheral T-cell lymphoma. The median age was 60 years and the male to female ratio was 1:1. A prior lymphoproliferative or autoimmune disorder was present in 14 per cent of the patients. Signs of advanced disease were usually present from the onset, such as B symptoms (55 per cent), generalized lymphadenopathy (57 per cent), stage III/IV disease (62 per cent), and elevated levels of serum lactate dehydrogenase (68 per cent). Primary extranodal disease (14 per cent), hepatomegaly (12 per cent), splenomegaly (12 per cent), lung/pleural involvement (12 per cent), skin involvement (21 per cent), and bone marrow involvement (28 per cent) were uncommon. Lymphocytopenia was present in 64 per cent of the patients, and none of nine patients tested were serologically positive for human T-cell leukemia/lymphoma virus (HTLV-I) infection. Among 38 patients receiving combination chemotherapy, 20 (53 per cent) achieved a complete remission. The actuarial median survival of all patients was 17 months. Age greater than 60 years and stage III/IV disease predicted a poor clinical outcome, whereas the large cell histological subtype predicted a favourable outcome. Prospective clinical studies using uniform treatments and a uniform histologic classification scheme are needed to confirm these findings.
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PMID:Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. 288 15

Lymphoplasmacytoid (lpc) and small cell centrocytic (scc) lymphoma are the two major sub-types of diffuse low grade non-Hodgkin's Lymphoma (NHL) within the Kiel classification. The presentation features and outcome for all 112 patients with these diagnoses (60 lpc, 52 scc) managed at St Bartholomew's Hospital between 1972 and 1986 are presented. The outcome for these patients is compared with that for patients with follicular and high grade lymphomas managed at this hospital during the same period. Nineteen of the 112 patients had localized (stages I-IIE) disease. In 18 of these cases the primary site of disease was extranodal, the gastrointestinal tract being involved in 12 cases. The survival for patients with localized disease was excellent. Eighteen are currently alive with median follow-up of 8 years. Ninety-three patients had advanced disease. A high incidence of splenomegaly, hepatomegaly, bone marrow and peripheral blood involvement was observed in both histological subgroups. A monoclonal paraprotein band was detected in the serum of nearly 50 per cent of patients with advanced lpc lymphoma. Patients with advanced disease were treated with either chlorambucil or cyclophosphamide, vincristine and prednisolone (CVP). The outcome was similar for both histological groups. Survival for these patients was poor (median 40 months) with less than 20 per cent surviving 5 years. Advanced age, elevated aspartate transaminase and failure to respond to treatment were identified by multivariate regression analysis as adverse prognostic factors.
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PMID:Lymphoplasmacytoid and small cell centrocytic non-Hodgkin's lymphoma--a retrospective analysis from St Bartholomew's Hospital 1972-1986. 290 57

T-cell chronic lymphocytic leukemia (T-CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T-CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E-rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T-cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin-2 production and lacked antibody-dependent cell-mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X;14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T-CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T-CLL, adult T-cell leukemia/lymphoma, the cutaneous T-cell lymphomas, and B-CLL.
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PMID:T-cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: a distinct clinicopathologic subtype with a poor prognosis. 293 74

We reviewed 82 cases of erythroderma, including clinical, laboratory, and biopsy material. The diseases most commonly associated with erythroderma were drug eruptions (34%), preexisting dermatoses (30%), and lymphoreticular neoplasms (20%), especially cutaneous T cell lymphomas (CTCL). The prognosis was related to the associated process; all deaths occurred in the drug or lymphoma groups. The clinical presentation of erythroderma was fairly uniform, with malaise (34%), pruritus (36%), and a subjective chilly sensation (34%) the most common symptoms. Keratoderma of the palms and soles (37%), mild generalized adenopathy (26%), and hepatomegaly (9%) were also common. Splenomegaly was rare (1%). Skin biopsy revealed the cause in 43% of the patients, but lymph node biopsy was not generally helpful in the initial evaluation. Although numerous laboratory values were abnormal, most findings were nondiagnostic and were related to the inflammatory process. A major exception was the Sezary cell preparation, which was selective for CTCL. A previously undiagnosed chronic erythroderma in a patient with no underlying skin disease may be the initial manifestation of CTCL. Thus, repeated evaluations and close follow-up are recommended.
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PMID:Erythroderma: review of 82 cases. 294 58

We report two cases of a T cell lymphoproliferative disease not previously described, with cytologic and clinical features similar to those associated with Galton's "prolymphocytic" leukemia (PL). Our patients, like those with Galton's PL, had massive splenomegaly and minimal or absent hepatomegaly and lymphadenopathy. In contrast, however, our patients had leukopenia, as well as low percentages of leukemic cells in the peripheral blood and in the bone marrow. In splenic imprints, the nuclear chromatin pattern of most of the leukemic cells was intermediate between those of mature lymphocytes and those of lymphoblasts, and the nuclei contained single, centrally located, conspicuous nucleoli. In sections of the spleen, the leukemic cells diffusely infiltrated the red pulp in a pattern strikingly similar to that of hairy cell leukemia; however, when the leukemic cells were studied cytochemically, the cytoplasmic acid phosphatase positivity was punctate and tartrate-sensitive. The leukemic cells were sheep erythrocyte rosette-positive and expressed T cell-associated antigens. Initially, both patients responded well to therapeutic splenectomy. One patient received combination chemotherapy after splenectomy and is alive and well 24 months after diagnosis. The other patient was in complete clinical remission for one year after splenectomy and received chemotherapy at relapse. He died, however, 23 months after splenectomy, with disseminated disease. IgG antibody titers against human T lymphotropic virus type I (HTLV-I) were detected in one patient and against HTLV-II in the other. The leukemia in these patients represents a distinct clinicopathologic entity within the spectrum of peripheral T cell lymphoproliferative diseases that includes Galton's PL of T cell derivation, T cell chronic lymphocytic leukemia, T cell hairy cell leukemia, and adult T cell leukemia/lymphoma.
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PMID:Leukopenic chronic T cell leukemia mimicking hairy cell leukemia: association with human retroviruses. 300 37

Virus associated adult T-cell leukemia/lymphoma (ATLL), which includes both adult T-cell leukemia (ATL) and its non-leukemic counterpart (NLATL) was studied clinically, histologically, and immunologically. The disease usually occurred in the sixth decade in both sexes equally. The patients had a rapid clinical course with frequent leukemic changes, lymphadenopathy, hepatomegaly, and occasional skin rash. Bone marrow involvement with mild infiltration and hypercalcemia were more frequent in ATL than in NLATL. Histologically the disease was categorized as malignant lymphoma, diffuse pleomorphic type with cerebriform nuclear giant cells. The lymphoma was characterized by diffuse proliferation of tumor cells with irregular nuclear configurations, varying in size and shape, and the presence of giant cells with highly convoluted cerebriform nuclei. The giant cells seemed to be a diagnostic marker. Immunologically, the tumor cells usually possessed the surface antigens recognized by OKT 3, 4, Leu 8 and anti-Tac antibodies, indicating that they were lymphomas of helper/inducer peripheral T-cells with the receptor for interleukin 2, but they demonstrated no helper/inducer functions. The patients often died of opportunistic infections due to T-cell dysfunction caused by the disease itself and strong chemotherapy.
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PMID:Virus associated adult T-cell leukemia (ATL) in Japan: clinical, histological and immunological studies. 300 99

Liver disease, although usually asymptomatic, is a frequent accompaniment of AIDS. Hepatomegaly and macrosteatosis are prevalent but non-specific findings. Evidence of remote hepatitis B virus infection is extremely common; however, the HBsAg carrier state, chronic active hepatitis, or cirrhosis occur no more frequently in AIDS patients than in the general population. Opportunistic intrahepatic infections (such as MAI, fungi, and CMV) or neoplasms (such as lymphoma or KS) usually reflect a disseminated process; liver involvement generally does not directly cause morbidity or result in death. Although biochemical liver tests are commonly elevated in the AIDS population, alkaline phosphatase has proved to be the most specific enzyme for infiltrative processes. Percutaneous liver biopsy has a high diagnostic yield, although the treatment options are currently limited. Acalculous cholecystitis and biliary tract obstruction have been recently described and probably result from CMV and/or cryptosporidial infection. Radiologic features of papillary stenosis and/or sclerosing cholangitis have been demonstrated. In contrast to hepatic parenchymal disease, these entities may be amenable to surgical or endoscopic therapeutic maneuvers.
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PMID:Hepatobiliary abnormalities of AIDS. 304 66

Cellular proliferation and differentiation of the mammalian mammary gland requires a medley of hormones including the anterior pituitary hormone, PRL. Recent evidence extends the role of PRL as a mammalian mitogen to cells in several physiological systems not directly involved in reproductive functions, such as liver and lymphocytes. PRL administration induces biochemical markers expressed during the G1 phase of cell cycle and activates DNA synthesis in rat liver. Chronic PRL treatment causes hepatomegaly, reflecting its stimulation of the proliferative process. In vitro, a lactogen-dependent cell line, the Nb2 rat node lymphoma cell, serves as a useful paradigm to study PRL action on mitogenesis. These cells, when cultured in the presence of lactogens, proliferate in a dose-dependent manner. The effects of various pharmacological agents on discrete phases of the cell cycle may be readily assessed in these cells since PRL-stimulated entry into cycle is signalled by an elevation of ODC activity at 6 hr and entry into S-phase at 6-12 hr. The parallel effects of phorbol ester tumor promoters and PRL on cell cycle progression in Nb2 lymphoma cells and in hepatic proliferation suggest that PRL may likewise mediate proliferation in aberrant growth conditions such as neoplasia. The data presented support the hypothesis that PRL is capable of promoting hepatocarcinogenesis. Its chronic administration after a hepatic initiating agent stimulated the development of histochemical and biochemical markers characteristic of preneoplasia. Further, the effect of PRL was comparable to that of the hepatocarcinogen when either was administered alone. Thus, hyperprolactinemia may serve to promote the development of hepatic tumors. Phorbol esters are thought to promote tumorigenesis by directly activating PKC. In the Nb2 lymphoma cell model, tumor promoting phorbol esters mimic the effects of PRL. Similarly, PRL-stimulated enzyme induction in liver is mirrored by phorbol ester treatment, and inhibitors of PKC block PRL-stimulated mitogenesis in Nb2 cells. Further, PRL or TPA administration to rats causes translocation of PKC activity from the hepatic cytosol to the membrane fraction, reflecting kinase activation. Therefore, PRL activation of PKC appears to be a physiological phenomenon of general significance, occurring as the result of lactogen receptor stimulation and serving to transmit intracellular signals linked to the regulation of mitogenesis. Further study is required to more fully define the scope of PRL-mediated mitogenic actions as well as its effects on the expression of differentiated products in tissues and cells.
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PMID:Prolactin as a mammalian mitogen and tumor promoter. 325 Feb 31

Eight cases of AIL-type T-cell malignant lymphoma are reported. The clinical symptoms are the same as those described in AIL: fever, malaise, weight loss, skin rashes, polyadenopathy, and splenomegaly. However, some differences can be noted: the absence of hepatomegaly in all cases but one, the absence of polyclonal hypergammapathy in all cases but one, and predominance in females. The lymph node modifications comprise diffuse infiltrations of lymphoid cells with irregular nuclei and pale cytoplasm, associated with a large number of immunoblasts and plasma cells. Some eosinophilic granulocytes and epithelioid cells can be seen. Hyperplasia of the vessels and remnants of follicles, sometimes with proliferation of follicular dendritic cells, are prominent features. The immunolabelling study demonstrates the presence of an important T-cell population all expressing a high predominance of CD 4 phenotype. These findings are in accordance with those published in Europe and in contrast with those of some of the Japanese cases, particularly the first patients published by Shimoyama et al. The differential diagnosis with AIL is based on the presence of clusters of mainly large cells with a pale cytoplasm, on the loss of expression of one T cell marker, as in 3 cases of our series, and on the presence of a high percentage of lymphoid cells engaged in the mitotic cycle as demonstrated with the Ki 67 monoclonal antibody. However, to draw a clear cut difference between AIL-type T-cell lymphoma and AIL considered as a prelymphomatous dysimmune lymphadenopathy, only the demonstration of cytogenetic abnormalities, as in one of our cases or of rearrangement of the genes coding for beta and/or gamma chain of the antigen receptor of T-cell are valuable criteria. The follow-up of our series is not long enough to appreciate the prognosis. Three patients died, one from a glioma. All the other cases, treated with polychemotherapy show total remission with an evolution of 10 to 39 months.
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PMID:Angio-immunoblastic lymphadenopathy (AIL) or T-cell malignant lymphoma of AIL-type. A histopathological, immunohistochemical and ultrastructural study of 8 cases. 326 11

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


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