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 45-year-old male was hospitalized on September 2, 1989 with chief complaints of general fatigue and fever. Physical examination revealed hepatomegaly and massive splenomegaly. Laboratory tests on admission showed Hb of 7.5g/dl, PLT 4.8 x 10(4)/microliters and WBC 9,610/microliters with 81% hairy cells. Bone marrow aspirate demonstrated 55.1% hairy cells and moderate myelofibrosis. Cytochemically, hairy cells were positive for tartrate-resistant acid phosphatase (TRAP). Surface markers were SmIg G+ A+ kappa +, CD11b+, CD11c+, CD19+, CD20+, CD21-, CD25+, HC2+, HLA-DR+. From these findings, a diagnosis of hairy cell leukemia (HCL) was made. After administration of deoxycoformycin (DCF) at a dose of 5.0mg/m2 1-2 times monthly, splenomegaly disappeared, as did hairy cells from the peripheral blood. Hematological level returned to within normal range except for the presence of 1.2% hairy cells and mild myelofibrosis in bone marrow aspirates. DCF has so far been effective for this patient. While DCF has been reported to be effective in the treatment of HCL in the West, it has not been determined in Japanese patients with HCL, who have different hematologic features from those of HCL patients in the West.
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PMID:[Hairy cell leukemia successfully treated with deoxycoformycin]. 146 84

Nineteen patients with hairy cell leukemia were studied in order to define the hepatic changes in this disease and to correlate the morphologic changes in the liver with the clinical and biochemical findings. Although only eight of the patients had hepatomegaly, all 19 had microscopic mononuclear cell infiltration in the sinusoids or the portal areas or both. The severity of mononuclear infiltration in the liver correlated poorly with the size of the liver or spleen, the biochemical changes, or number of hairy cells in the blood. Abnormal serum biochemical values were present occasionally and were usually due to associated diseases or to complications of this disease. Elevated serum alkaline phosphatase activity was noted in four patients; three of them had granulomatous lesions in the liver. Unless the characteristic "clear cell" pattern is seen, the hepatic mononuclear cell infiltration may not be diagnostic of hairy cell leukemia and, in many instances, may not even be suggestive of neoplasia. A new technique for demonstrating tartrate-resistant acid phosphatase activity in methacrylate-embedded sections was developed, which allowed identification of hairy cells in the liver biopsy specimens of all five patients so studied. The authors concluded that liver involvement is common in this disease.
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PMID:Hepatic involvement in hairy cell leukemia. 682 56