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)

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.
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PMID:[Primary myelofibrosis: description of a series of 53 patients]. 266 65

A 68-year-old man was referred to our hospital because of generalized lymphadenopathy. His abnormal findings were hepatomegaly, leukocytosis and elevated serum LDH. Anti-ATLA antibody was positive. As about half of the peripheral lymphocytes reacted to the monoclonal antibody CD25, these cells were considered to be compatible with ATL cells having an interleukin-2 receptor. Initially, recombinant beta-interferon was administered, but it was not effective. A combination of vincristine (VCR) and prednisolone (PDN) was partially effective against the lymphadenopathy, but the hepatomegaly and leukocytosis did not improve. 4'-epi-Adriamycin (Epirubicin), at a doses of 20 mg/body, weekly, was subsequently added to VCR + PDN therapy. The patient achieved complete regression 2 months later, and has been in continuous complete remission for more than 3 months.
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PMID:[A case of adult T-cell leukemia achieving complete remission with epirubicin, vincristine and prednisolone chemotherapy]. 288 35

An autopsy case of ATL with acute hepatic failure is reported. A 39-year-old man born in Miyazaki Prefecture was admitted because of jaundice and general malaise of about 10 days' duration. Palpation of the abdomen revealed moderate hepatomegaly: the patient had clear consciousness. The white cell count was 62,500/mm3, with 34% abnormal lymphocytes having lobulated or convoluted nuclei. Marked liver dysfunction (TB 30.5 mg/dl; GOT 2,740 U; GPT 1,031 U; LDH 3,833 U) was noted. He developed hepatic coma and died of acute hepatic failure on the third hospital day. At autopsy, the enlarged liver weighed 2,000 g and was diffusely infiltrated by ATL cells.
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PMID:[An autopsy case of adult T-cell leukemia with acute hepatic failure]. 301 18

Interferon-beta (GKT-beta) was administered to a patient with adult T-cell leukemia (ATL), presumably of acute type, with a marked organ infiltration and increases in serum calcium, LDH and bilirubin. The therapy produced a fall in the number of peripheral blood abnormal lymphocytes, disappearance of jaundice, reduction in the degree of hepatomegaly and splenomegaly and a remarkable improvement of general condition with a performance status of grade 3 improving to grade 1. The response has been maintained for more than seven months. Dose, schedule and criteria for drug discontinuation are matters for further investigation.
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PMID:[A case of adult T-cell leukemia responding to recombinant interferon-beta (GKT-beta)]. 348 33

A 66-year-old man was admitted for shortness of breath and showed fever, abdominal fullness and paraplegia. Monocytosis amounting to 25% and an elevation of serum LDH (4,281 mIu), were remarkable in the laboratory findings. He died of pulmonary insufficiency about a month after admission. On autopsy hepatomegaly (1950 g), splenomegaly (780 g), but no lymphadenopathy and small infarction in the thoracic spinal cord causing paraplegia was noted. Histopathologically, the invasion of the tumor cells into the liver, spleen, lymph nodes, bone marrow and other organs was observed. Malignant histiocytosis was diagnosed by histologic and immunohistochemical studies (lysozyme positive, S-100 protein negative).
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PMID:[A case of malignant histiocytosis with paraplegia]. 362 41

Young rats were force-fed a lysine + arginine-devoid diet or a complete diet for 3 days, and selected biochemical and morphologic studies were conducted. Rats force-fed the experimental diet in comparison with those force-fed the control diet for 3 days showed decreased body weight gain, hepatomegaly with periportal fatty liver, pancreatic and splenic atrophy, and enhanced 14C-leucine incorporation into hepatic proteins. Differences in the experimental animals were observed in the free amino acid levels of serum (decreased lysine, arginine, and ornithine) and liver (decreased ornithine), in blood chemistries (decreased levels of ammonia N2, uric acid, cholesterol, protein, albumin, alkaline phosphatase, LDH and SGOT) and in hematologic findings (leukocytopenia and thrombocytopenia after a morning feeding). The experimental findings in young rats force-fed the lysine + arginine-devoid diet were compared with those reported to develop in children with lysinuric protein intolerance (LPI), an autosomal recessive defect in diamino acid transport. Children with LPI as described by others reveal a number of similarities as well as a number of differences in comparison to the findings in the experimental animals. The comparison suggests that some of the pathological manifestations of LPI may be related to a deficiency of diamino acids but others must be due to different alterations in this complex human disease.
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PMID:Chemical pathology of diamino acid deficiency: considerations in relation to lysinuric protein intolerance. 393 96

Among 2175 patients seen over the last three years in a non-specialized department of internal medicine with no intensive care unit, 100 had supranormal serum lactic dehydrogenase activities. These patients' case-reports have been analyzed. Nearly half the patients (47/100) had a malignant disease (cancer or hemopathy). Among the remaining patients, 19 had a hepatic disorder (alcohol hepatitis in 10, viral hepatitis in 8, and isoniazide hepatitis in 1), 7 had a heart disease (heart failure with hepatomegaly in 5, myocardial infarction in 2), and 27 had various other conditions (including hemolysis in 6 and polymyositis en 3). The value of serum LDH assay is obvious in situations other than acute conditions such as myocardial infarction of pulmonary embolism; these are better known and have not been studied here as their prevalence was low among the patients enlisted in our study. In comparison to other enzymes (alkaline phosphatase (AP), gamma-glutamyl transpeptidase (GGT), transaminases (GOT, GPT) that were also routinely assayed in our patients, abnormal serum LDH activities are much less common and their significance is quite different. An increase in serum and their significance is quite different. An increase in serum LDH activity indicates a serious condition, often with a fatal outcome. The "various other conditions" group includes patients with hemolysis, hepatitis and myositis; the other patients in this group either had severe infectious diseases or died suddenly in the first few days of their hospitalization before diagnosis had been established. Each etiologic group has been analyzed to asses the characteristics of patients with increased LDH activity according to each etiology. Analysis of coincident abnormalities of the other enzymes listed above shows marked differences between etiologic groups; diagnostic accuracy can thus be enhanced in certain conditions. Most patients with malignancies had poorly differentiated tumors, with metastases: 28 had an epithelial tumor, with hepatic and/or bone metastases in 23 cases, 5 had cancer of the liver, 10 had a malignant hemopathy (2 lymphomas, 5 myeloproliferative syndromes, 3 acute leukemias), and 4 had a sarcoma. Cancer of the lung is the most common malignancy (10 cases) and may be responsible for increased serum LDH activity even in patients without metastases. Serum LDH assay is of value for monitoring the course in patients with initially increased activities as it falls under effective therapy and rises during exacerbations.
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PMID:[Value and diagnostic significance of serum lactic dehydrogenase in internal medicine (author's transl)]. 628 24

Two cases of peliosis hepatis, treated with a large dose of predonine for aplastic anemia and multiple myeloma, respectively, were reported. Case 1 showed no abnormal liver function, but, in Case 2 hepatomegaly, retention of ICG, and elevation of LDH level despite the normal serum transaminase were noted. In both cases, peliotic lesions were incidentally observed at autopsy. In Case 1, the lesion was localized in the right lobe only, but, in Case 2 the lesion was present throughout the entire lobes of the liver involving also a part of the spleen and bone marrow of the sternum. Innumerable blood-filled cavities were surrounded by incomplete reticulin or injured hepatocytes. In smaller lesions observed in Case 1, focal necrosis, hemorrhage, inflammation, and thrombi were prominent features. On the other hand, in extensive lesions, as in Case 2, a marked liver cell dissociation with sequent irregular reticulin arrangement was present. Though the etiology and peliosis hepatis is still uncertain in these cases, it was assumed that a large dose of predonine might have some correlation to the occurrence.
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PMID:Peliosis hepatis. Report of two autopsy cases with a review of literature. 713 99

We reported a rare case of malignant lymphoma, highly suspected to be of pancreas origin with multiple nodular hepatic invasions. A 63-year-old man visited our hospital on May 24 1990, complaining of anorexia. In spite of high serum levels of LDH and amylase, abdominal ultrasonography (US) showed no significant lesions. Forty days later, he was admitted because of the loss of weight and the appearance of abdominal pain with marked hepatomegaly, exhibiting cachexia. Abdominal US and computed tomography visualized a tumor of pancreas with multiple nodular space-occupying lesions in the liver. In several days, jaundice and ascites appeared with progressive deterioration. Angiography showed no tumor vascularity nor vascular stain in pancreas and liver, suggesting a non-epithelial malignancy. Mitomycin C and Epi-adriamycin, given to the patient via the celiac artery, brought about a remarkable shrinkage of the abdominal tumor. Subsequent systemic chemotherapies and occasional intra-arterial chemotherapies resulted in the disappearance of abdominal tumor clinically. However, at the end of April 1991, signs of CNS involvement were observed, and he died on June 7, 1991. Autopsy revealed that malignant lymphoma, of diffuse large cell type, had invaded the subarachnoidal cavity without any evidence of invasion of the abdomen or other sites except regional necrotic lesion in the pancreas. These findings strongly suggested that the patient suffered from malignant lymphoma of pancreas origin with multiple nodular hepatic invasions.
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PMID:[A case of malignant lymphoma, highly suspected to be of pancreatic origin, with multiple nodular hepatic invasions]. 794 2

A total of 41 non-cutaneous peripheral T-cell lymphomas were classified following the updated Kiel classification. Of these, 20 cases belonged to the low-grade group (T-cell chronic lymphocytic leukaemia, 3; lymphoepithelioid, 5; angioimmunoblastic, 4; pleomorphic small cell, 8) and 21 to the high grade group (pleomorphic medium and large cell, 11; immunoblastic, 3; large-cell anaplastic Ki-1 positive, 7). Seventy per cent showed a CD4+/CD8-phenotype, 39% a defective phenotype and 88% an activation phenotype. Eighty per cent had B-symptoms, 63% hepatomegaly, 48% splenomegaly and 26% had involvement of more than three lymphoid areas. Bone marrow was infiltrated in 34%, central nervous system in 4%, lung in 12% and skin in 14.6%. Seventeen per cent presented with extranodal disease and 82.8% had stage III/IV disease. Hypergammaglobulinaemia was found in 29%, hypercalcaemia in 7%, raised LDH serum levels in 58% and HTLV-I antibodies in only one case. Of the 37 treated patients 18 (48%) achieved a complete remission, but 33% relapsed. Mortality was 59% and actuarial overall survival at 38 months was 0.32. In the comparison of the clinical, analytical and immunophenotypic variables and outcome between low and high grade groups, only the average of bone marrow infiltration in the low grade and stage I-II, presence of defective phenotypes and higher Ki-67 positivity in the high grade group were significantly different.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Peripheral T-cell lymphoma: a clinicopathological study of 41 cases and evaluation of the prognostic significance of the updated Kiel classification. 851 73


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