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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Erythrokinetics were studied in 29 patients with hairy-cell leukaemia. In all cases there was an increase in plasma volume, closely correlated to the size of the spleen, indicating that the true degree of anaemia can only be appreciated by red cell volume measurement. Moderately increased haemolysis was observed in most cases, which did not correlate with the spleen size. Simultaneous study of autologous and isologous red cell life-span suggested an extra-corpuscular mechanism for the haemolysis in most patients. A quantitative erythropoietic defect, either relative or absolute, was found in half the cases, without any qualitative defect. Only one case showed erythroid metaplasia of the spleen. Thus marrow failure appears to be largely responsible for the anaemia and granulocytopenia in hairy-cell leukaemia. A clear correlation was shown between the short-term prognosis after splenectomy and the degree of hypersplenism. However, long-term survival correlated chiefly with the degree of bone marrow failure, whether splenectomy had been carried out or not. The results indicate that isotope studies in hairy-cell leukaemia are useful both in determining the best form of treatment and predicting survival.
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PMID:Erythrokinetic studies in hairy-cell leukaemia. 46 64

A number of the 'monocytic' features of the hairy cells (HCs) of hairy-cell leukemia (HCL) are studied in detail and discussed in relation to the now established B-cell nature of the disease. By means of a rosette assay employing erythrocytes coated with IgG (EAG), a receptor for the Fc of IgG is shown to be strongly expressed on all HCs, whether in suspension or in monolayers. This receptor is shed and re-expressed over a period of some 6 h. In EAG rosette formation the indicator erythrocytes are deformed and the receptor is not blocked by an antiserum to the Ia-like antigen. By ultrastructural acid-phosphatase cytochemistry, it is established that HCs phagocytose latex particles, but do not phagocytose a variety of other particles to a significant extent. These, and other features, are discussed in relation to the literature and it is shown that all are compatible with the current view that HCL is a form of B-cell lympholiferative.
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PMID:A re-examination of the alleged monocytic features of hairy-cell leukaemia. 47 61

The hairy-cells (HC) of 10 patients with hairy-cell leukaemia were studied with several techniques to evaluate their phagocytic potential. Mononuclear cells from normal donors and from patients with acute monocytic leukaemia served as controls. Light microscopically HC seemed to have ingested bacteria or latex particles. Treatment of the cells with lysostaphin, an enzyme that kills extracellular Staphylococcus aureus, showed that almost all 'ingested' bacteria were extracellular. Lanthanum nitrate, added during the fixation procedure for electron microscopy, stained both the outer cell membrane and the membranes of the 'phagosomes' of the HC, also indicating that the 'ingested' particles were extracellular. HC showed no increased oxygen consumption on exposure to bacteria in the presence of serum. Furthermore, HC showed no lysozyme or peroxidase activity, whereas non-specific esterase activity was much weaker than in monocytes. These findings, which show that HC are essentially non-phagocytic, constitute strong evidence against a monocytic origin of the malignant cells of hairy-cell leukaemia.
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PMID:Phagocytic potential of hairy cells. 49 73

A case of hairy-cell leukaemia (HCL) with IgG(k) paraprotein is described. The typical clinico-pathological features of the patient are stressed, and it is shown that the hairy cells (HCs) were producing and secreting IgGk. The case is placed in a more general context by the demonstration that HCs from other typical cases of HCL produce and secrete IgG. These findings conclusively demonstrate that HCL is a B-cell disease.
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PMID:Typical hairy-cell leukaemia with IgGk paraproteinaemia. 50 31

We analysed by liquid scintillation counting and by autoradiography the DNA repair synthesis induced by UV irradiation in mononucleated cells from peripheral blood of 'hairy cell leukaemia' patients. In 9 out of 12 patients the repair activity values obtained in repeated assays were found to be significantly lower than those of healthy donors.
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PMID:UV-induced DNA repair in hairy cell leukaemia patients. 50 6

A quantitative study of various aspects of platelet function was carried out in eight patients with typical hairy-cell leukaemia (HCL). In at least two patients platelet aggregation was convincingly reduced to more than one aggregating agent (ADP, adrenaline, collagen, thrombin, and ristocetin). Granular storage capacity for {(14)C} 5-HT was reduced in five of the six patients tested. The two patients with definitely abnormal aggregation had the greatest reduction in granular storage pool and the longest bleeding times of those tested but, like the other patients, they did not have a clinical haemostatic defect. It was concluded that a granular storage pool defect (SPD) was at least partly responsible for aggregation abnormalities in HCL since the platelet release reaction in response to thrombin appeared to be normal. All our patients ran a chronic course uncomplicated by any of the factors known to predispose to a platelet SPD acquired in the circulation. Although in the one patient tested before and after splenectomy there was some improvement in platelet aggregation after operation, there was no clear general relationship between defective platelet function and either previous splenectomy or platelet count. Since a direct involvement of the megakaryocytic series in the underlying cell proliferation of HCL seems unlikely, it is concluded that the platelet defect can most reasonably be attributed to the production of abnormal platelets as a result of marrow fibrosis and/or infiltration by hairy cells.
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PMID:Platelet function in hairy-cell leukaemia. 51 41

The peripheral blood cells, spleen cells and bone marrow cells from a patient with hairy cell leukaemia were studied by means of several immunological methods and by phase contrast and electron microscopy. Both by light and electron microscopy the cells had the morphology of hairy cells. 60 % of all the peripheral blood cells, and 80 % of the spleen cells had membrane-bound IgGk immunoglobulin. 60 % of the peripheral blood lymphocytes and 90 % of the spleen cells were positive for Ia-antigens, and 80 % of the peripheral blood, and 70 % of the spleen cells had receptors for complement factor C3. The percentages of cells with receptor for the Fc part of IgC and receptors for sheep red blood cells (SRBC) were low both in peripheral blood and in spleen. Reduced numbers of peroxidase positive cells and cytotoxic plaque-forming cells were also observed as well as reduced lymphocyte responses after stimulation of peripheral blood lymphocytes with PHA, PWM, ConA, PPD and allogeneic cells. A normal antibody-dependent cell cytotoxicity (ADCC) and PHA-induced cytotoxicity was observed for the peripheral blood lymphocytes of the patients. Our results suggest that the hairy cells in our patient are derived from B lymphocytes and have a monoclonal origin.
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PMID:Characterization of peripheral blood, spleen and bone marrow cells from a patient with hairy cell leukaemia. 54 2

In 82 patients with non-Hodgkin lymphoma (NHC) the DNA synthesis by mononuclear cells from the peripheral blood was assessed by means of the index of mitoses (IM) or by pulse labelling of cells with 3H-TdR. In chronic lymphatic leukaemia (47 cases), hairy-cell leukemia (1 case), plasma-cell leukaemia (1 case) no synthesis of DNA was found in mononuclear cells. On the other hand, it was raised in most cases of lymphoplasmocytoma, centrocytoma, centroblasto-centrocytoma, centroblastoma and in lymphoblastic leukaemia or lymphoma.
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PMID:[Mononuclear peripheral blood cells in patients with non-Hodgkin's lymphoma synthesizing DNA in vitro]. 71 94

Twenty-six patients with hairy cell leukemia have been evaluated clinically at Stanford University Medical Center and the M. D. Anderson Hospital and Tumor Institute since 1973. Only four patients had obvious leukemia and readily identifiable hairy cells in the peripheral blood. The remaining patients were pancytopenic, and hairy cells in peripheral blood were difficult to recognize. In 20 of the latter patients the marrow aspirates were unsuccessful or nondiagnostic. Bone-marrow biopsy was the primary method of diagnosis in 18 cases and proved the most reliable and pertinent diagnostic procedure in identifying this disorder. A well-defined water-clear rim of cytoplasm surrounding and separating the hairy cell nuclei was the main histologic feature, in contrast to the lack of visible cytoplasm and close appositon of lymphocytes in chronic lymphocytic leukemia or poorly differentiated lymphocytic lymphoma. The uniformity and blandness of the nuclei distinguished hairy cells in tissue sections from the cells of histiocytic lymphoma and acute myeloid leukemia. Awareness of the importance of the histologic appearance of the bone-marrow biopsy in hairy cell leukemia is essential in establishing an accurate diagnosis.
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PMID:The value of the bone-marrow biopsy in the diagnosis of hairy cell leukemia. 72 72

A case of hairy cell leukaemia (HCL) treated by single-agent chemotherapy after the failure of splenectomy is reported. The patient was treated with doxorubicin 50 mg on 4 occasions over an 8-day period. After 2 weeks of profound pancytopenia, substantial blood and bone marrow improvement ensued. This case demonstrates that HCL may in some case be treated successfully by intensive chemotherapy.
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PMID:Response to doxorubicin in hairy cell leukaemia. 73 54


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