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)

Hairy-cell leukaemia (leukaemic reticuloendotheliosis) is a well-defined clinical entity. Most of the recent reports are almost entirely concerned with the pathological and functional aspects of the disease. In the present retrospective study the clinical features and laboratory data of 12 patients were analyzed together with a series of 123 adequately clinically documented cases from the literature. The Hb level and the sex of the patient proved to be the only parameters having some prognostic value for the survival time after diagnosis. The effect of splenectomy was assessed in two comparable groups of 24 splenectomized and 51 non-splenectomized patients. The operation seemed to be beneficial, but after 2 years the difference was not significant(.05 less than P less than .10). Analysis of subgroups showed that splenectomy was definitely beneficial in women, in patients with a Hb level over 8.0 g/dl or a platelet level above 50 X 10(9)/1, in patients with leucocytes below 3 X 10(9/1, , and also in patients with hepatomegaly (P less than .05 in all cases). These findings suggest that splenectomy is beneficial in cases where anaemia and thrombocytopenia are not very severe; in severe cytopenia the operation does not increase the life expectancy.
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PMID:Hairy cell leukaemia. Clinical features and effect of splenectomy. 69 19

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

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

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

Fifty-two patients with different types of leukemia [27 chronic lymphocytic leukemia (CLL), 10 acute myeloblastic leukemia (AML), eight acute lymphoblastic leukemia (ALL), four chronic myelogenous leukemia (CML), and three hairy cell leukemia (HCL)] had abdominal and pelvic CT. The radiographic features are reviewed to evaluate the role of CT in diagnosis and management of these patients. The highest incidence of lymphadenopathy was observed in CLL followed by HCL and ALL. Other findings included splenomegaly, hepatomegaly, ascites, pleural effusion, microabscesses in liver and kidney, and visceral involvement, i.e. intestine, bladder, and bone.
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PMID:Abdominal and pelvic computed tomography in leukemic patients. 659 May 76

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

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

HCL typically presents in middle-aged men, and is characterized by splenomegaly and cytopenias. Hepatomegaly may be present, but it usually is not a salient feature. Peripheral adenopathy is uncommon. Other organ manifestations occur, but are unusual. patients are now presenting with a less tumor burden, as a result of earlier diagnosis. Leukocytosis/lymphocytosis should suggest HCl variant. Infectious complications, which were common in the past and the major cause of death, have become rare in the era of purine analog therapy. Whether there is a true increased risk for second malignancies remains controversial.
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PMID:Clinical presentations and complications of hairy cell leukemia. 1699 Jan 7

We report a 68-year-old Indian man who was referred to the Hematology Unit for investigation for thrombocytopenia, an incidental finding during a pre-operative screening for prostatectomy. Physical examination was unremarkable. There was no splenomegaly, hepatomegaly or lymphadenopathy. Complete blood counts showed normal hemoglobin and total white cell count with moderate thrombocytopenia. Hairy-cell leukemia was diagnosed based on peripheral blood film, bone-marrow aspirate and trephine biopsy findings, supported by immunophenotyping results by flow cytometry. The purpose of this report is to create awareness of this uncommon presentation and to emphasize that a single-lineage cytopenia or absence of splenomegaly does not exclude the diagnosis of hairy-cell leukemia. Careful attention to morphological detail is important for early diagnosis, especially when low percentages of "hairy" cells are present in the peripheral blood and bone marrow. Early diagnosis is important to ensure that patients obtain maximum benefit from the newer therapeutic agents that have greatly improved the prognosis in this rare disorder.
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PMID:Hairy-cell leukemia: a rare blood disorder in Asia. 1754 79


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