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)

A series of 27 patients with myelodysplastic syndrome (MDS) was analysed to determine the clinical and pathologic features, the natural history and the prognostic factors. The clinical features of these patients included the following: mean age 53.77 years; symptoms due to cytopenias 100 per cent; hepatomegaly 44.5 per cent; splenomegaly 11 per cent. Almost all patients with MDS presented anemia; additional cytopenias were present in many patients. The bone marrow was hypocellular in 1/3 of cases. The patients have been classified according to the French-American-British (FAB) Group criteria: there were 11 patients with refractory anemia, 11 patients with refractory anemia with sideroblasts and 5 patients with refractory anemia with excess of blasts. The mean survival was 19 months, 9 patients died: death was due to infection or hemorrhage in 7 cases and to evolution to acute myelogenous leukemia (AML) in 2 cases (10.5%). Thrombocytopenia, the Bournemouth scoring system and FAB classification were independent prognostic factors.
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PMID:[Myelodysplastic syndromes. A clinico-biological analysis of 27 cases]. 207 37

A 21-year-old woman presented with a 12-month history of epigastric pain, and for 3 months she had noticed a mass in the right hypochondrium. She had taken 'Norinyl-1' (norethisterone 1 mg and mestranol 50 mcg) for 5 years. She smoked 20 cigarettes a day but drank little alcohol. Physical examination revealed irregular hard hepatomegaly 10 cm below the right costal margin. Hepatitis B surface antigen was not detected in the serum and alpha fetoprotein levels were normal ( 10 M.R.C. units). A liver scan showed a large space-occupying lesion in the right lobe of the liver, and liver biopsy revealed a cholangicarcinoma with striking fibrous reaction. Multiple shadows consistent with metastases were present on chest X-ray, but no bony deposits were found on radiological skeletal survey or bone scan. The serum calcium was persistently high (2.74-2.92 mmol/l) but fell on prednisolone therapy. Serum parathyroid hormone levels were normal. A causal relation between oral contraceptives and hepatic adenoma is now generally accepted, and several patients with hepatocellular carcinoma have also been reported. We have been able to find only 1 previous report of cholangiocarcinoma in a young female taking oral contraceptives, and there is 1 report of this tumor in a man taking high doses of anabolic steroids for refractory anemia. This tumor has its peak incidence in the 6th decade and is very rare in the 3rd decade. The association with hypercalcemia due to pseudohyperparathyroidism is well recognized. In only some cases are parathyroid hormone levels raised, and the cause of the pseudohypercalcemia in our patient is unknown.
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PMID:Cholangiocarcinoma and oral contraceptives. 610 61

A 75-year-old woman presented with anemia, lymphadenopathy, hepatomegaly and lingual tumor, but no constitutional symptoms. The laboratory data showed pancytopenia and polyclonal hypergammaglobulinemia. A bone marrow aspirate represented an apparent myelodysplastic syndrome (MDS) feature, specifically, refractory anemia with excess of blasts. A lymph-node biopsy revealed the disappearance of normal architecture, small arborizing blood vessels, large lymphoid cells with prominent cytoplasm (so-called pale cells) and a clonal proliferation of T-lymphocytes. The patient was diagnosed as having MDS associated with immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma. She was subsequently treated with cyclophosphamide, adriamycin, vincristine and prednisolone for lymphoma which successfully induced a remission of not only the T-cell lymphoma but also the MDS. The case suggested that MDS might be a paraneoplastic complication of IBL-like T-cell lymphoma.
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PMID:Myelodysplastic syndrome associated with immunoblastic lymphadenopathy-like T-cell lymphoma: simultaneous clinical improvement with chemotherapy. 815 57