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)

Evaluation of the histologic changes of the bone marrow and clinical data of twelve male and two female patients with hairy cell leukamia before treatment. Mean age of the patients was 46 years, time from the onset of symptoms 5.5 months. 13 of the patients were anaemic, showing splenomegaly, 12 suffered from thrombopenia, 9 from granulocytopenia, and 8 from hepatomegaly. In all of the cases, the relative numbers of lymphocytes in the blood had been increased together with various amounts of characteristic hairy cells. All of the cases had a typical histologic picture of lymphocytic bone marrow infiltration, mostly of the diffuse type. In 50% of the cases not only the well known rod-like intracellular inclusions could be seen, but also ring-shaped figures whose significance is discussed. Decrease of the granulopoiesis, disintergration of the marrow sinusoids, and osteoporosis are the most important additional signs. The progression of the disease is marked by increasing bone marrow infiltration, by splenohepatomegaly, anaemia, thrombopenia, and increasing numbers of typical lymphocytes in the blood. The bone marrow being considered to be the origin of the disease for good reasons, the histobiopsy of this organ ranks among the diagnostic and prognostic measures to be taken at first sight.
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PMID:[Bone marrow diagnostic in hairy cell leukaemia (author's transl)]. 92 7

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

Clinicopathological findings of six cases of Hairy cell leukaemia are presented. All the patients were males, the age ranged between 32-57 years. Complications of anaemia and neutropenia were common modes of presentation. Hepatomegaly and splenomegaly were present in all the cases whereas only 2 patients had lymphadenopathy. Severe pancytopenia was detected in 3 cases and circulating hairy cells were present in all the cases. Trephine biopsy done in all six patients was found to be diagnostic. Tartrate resistant acid phosphatase was detected in the hairy cells of 2 cases.
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PMID:A clinico-pathological study of six cases of hairy cell leukaemia. 179 14

Vicia villosa (hairy vetch) is used as a forage source in some cattle-producing areas in Argentina. The plant had no previous reports of toxicity in this country. A herd of 33 Aberdeen Angus bulls grazed during 20 days in October on a pasture composed mainly of hairy vetch. Eight animals developed conjunctivitis, rinitis, dermatitis, loss of hair and fever. All of them died within 15 d after the development of signs with a marked loss of body condition. No more animals became sick 5 d after the removal of the herd from the pasture. Serum parameters tested (calcium, phosphorus, magnesium, GOT, alfa-GT and bilirubin) enlarged liver and spleen, generalized hemorrhage in the abomasum, dilated kidneys and multiple pale areas on the heart. Severe necrotizing granulomatous myocarditis, interstitial nephritis, and necrotizing cholangitis were the most striking microscopic changes. Close observation of animals feeding on pastures in which V villosa is dominant is the only prevention.
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PMID:An outbreak of Vicia villosa (hairy vetch) poisoning in grazing Aberdeen Angus bulls in Argentina. 185 12

A syndrome which is known as plasma cell dyscrasia with polyneuropathy and various endocrine manifestations or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein and skin changes is very interesting because this syndrome has miscellaneous manifestations such as skin hyperpigmentation, hypertrichosis, polyneuropathy, M protein abnormality, plasma cell dyscrasia and endocrine disturbances. Miscellaneous endocrine abnormalities which have not been described so far are reported here. A 47 year old female was admitted with the chief complaints of edema and gait disturbance. Past and family histories were noncontributory. In April 1981, edema appeared in her face and legs. In June she noticed paresthesia in her legs. Edema increased gradually and she had difficulty walking. Her skin became pigmented and hairy. In October she was admitted because of polyneuropathy with increased cerebrospinal fluid protein without pleocytosis. Prednisolone was started. Walking improved slightly, but edema and paresthesia remained unchanged. Prednisolone was stopped at the end of the following March. In May 1982, she was admitted for further evaluation of edema and polyneuropathy. The patient was alert and cooperative. On standing the skin of her legs became cyanotic. There was hypertrichosis on the arms and legs. Her fingers were clubbed. A moderate swelling of the cervical lymph nodes was noted. There was mild hepatomegaly without splenomegaly. All tendon reflexes were lost. Plantar response was flexor. Muscular strength diminished mildly. She complained of paresthesia on the soles. Superficial sensation was normal. Vibratory sense decreased mildly. Cerebellar function and cranial nerves were normal. There was no sphincter disturbance. The examination of urine, stool and peripheral blood was normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Marked endocrine abnormalities in polyneuritis with skin hyperpigmentation, hypertrichosis, edema and increased lambda type immunoglobulin A: a case report]. 641 99

An unusual patient with hairy cell leukemia (HCL) who developed marked hepatomegaly due to a large vascular tumor in the liver is reported. The relation of this vascular tumor to the microscopic splenic pseudosinuses and hepatic angiomatous lesions encountered in HCL is discussed. To the best of our knowledge this represents the first case report of the association of HCL with large macroscopic hemangioma of the liver causing hepatomegaly. The patient also developed a large paratracheal mediastinal mass with a recurrent pleural effusion which was shown to contain many typical hairy cells. This rare finding is discussed in relation to the isolated cases of lymphocytic lymphoma who present with clinical and morphological features mimicking HCL. This patient had HCL according to all established criteria with characteristic morphological, cytochemical and ultrastructural features and the pleural effusion and mediastinal mass were most probably part of the HCL neoplasia, despite the fact that biopsy was not performed.
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PMID:Hairy cell leukemia: report of an unusual case with hepatomegaly due to a large vascular tumor of the liver, mediastinal mass and pleural effusion containing hairy cells. 643 21

From 1972 to 1981 10 patients with hairy cell leukaemia were observed in the Medical University Clinic Cologne, this represents 1.8% of all leukaemias. Typical clinical signs are splenomegaly, no or only slightly enlarged lymph nodes and a moderate hepatomegaly. Almost in all cases an anaemia, thrombocytopenia and neutropenia with lymphocytosis was found, mostly combined as pancytopenia. The pathognomonic tartrate resistant acid phosphatase was found in the hairy cells to a differing amount besides a fibrosis and a lymphatic infiltration of the bone marrow. A normalization of the anaemia, the thrombocytopenia and the neutropenia was reached by splenectomy, but the increased susceptibility to infections could not be affected significantly.
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PMID:[Clinical aspects of hairy cell leukemia and its modification by splenectomy]. 676 22

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

Thirty-six patients with hairy-cell leukemia (HCL) were evaluated, and were divided in two major subtypes: leukopenic (WBC less than 3000/microliters) and non-leukopenic (WBC greater than or equal to 3000/microliters). There were 22 leukopenic and 14 non-leukopenic patients. The leukopenic group were older than the non-leukopenic group, with an average age of 58.4 years compared with 47.6 years. The male/female ratio was higher in the leukopenic (6.3) than the non-leukopenic (2.0) patients. Splenomegaly, hepatomegaly and lymphadenopathy were found in 66%, 32%, and 18% of the leukopenic patients, compared with 92%, 57%, and 35% in the non-leukopenic patients. The leukopenic HCL was associated with more severe anemia, granulocytopenia, monocytopenia, and thrombocytopenia, and higher incidence of serious infections than the non-leukopenic HCL. Increased bone marrow reticulin fibers and unsuccessful marrow aspirations (dry taps) were more frequently associated with the leukopenic than the non-leukopenic HCL.
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PMID:Clinicopathological subtypes in hairy-cell leukemia. 710 11

Hairy cell leukaemia variant is a very rare chronic lymphoproliferative disorder and is closely related to hairy cell leukemia. We hereby describe a case of hairy cell leukaemia variant for the first time in Saudi Arabia. An elderly Saudi man presented with pallor, massive splenomegaly, and moderate hepatomegaly. Hemoglobin was 7.7 g/dl, Platelets were 134 x109/l and white blood count was 140x10 9/l with 97% being abnormal lymphoid cells with cytoplasmic projections. The morphology, cytochemistry, and immunophenotype of the lymphoid cells were classical of hairy cell leukaemia variant. The bone marrow was easily aspirated and findings were consistent with hairy cell leukaemia variant.
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PMID:Hairy cell leukemia-variant. 1125 16


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