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

A case of a woman suffering of hairy cells leukemia (tricholeucocytes) is described. The diagnosis of this leukemia was set two years after a splemectomy, due to the enlargement of the spleen. The patient was treated for a period of two years as a refractory anaemia and leucopenia (neutropenia). After admission the diagnosis was confirmed by the examination of periferal blood, cytologic and histologic examination of the bone marrow and after a subsequent examination of the spleen tissue. All these examinations showed the presence of typical hairy cells--tricholeucocytes in the periferal blood, bone marrow and spleen. On account of their own cytochemical examinations, and especialy immunological examinations by other authors, the authors of this presentation suppose that the tricholeucocytes most probably belong to B lymphocytes and that, therefore, this leukemia should be placed in the group of malignant lymphocytoproliferative diseases.
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PMID:[Hairy-leukocyte leukemia (tricholeukocyte). Report of a case]. 108 56

Eight cases of 'hairy' cell leukaemia (leukaemic reticuloendotheliosis) were evaluated by scanning electron microscopy. The surface of critical-point-dried 'hairy' cells was characterized by prominent and exaggerated broad-based, ruffled membranes and scattered small clusters of stub-like microvilli. The surface morphology resembled that of normal and leukaemic monocytes, but differed from that of normal and leukaemic lymphocytes. Some cells with features of both lymphocytes and monocytes were difficult to categorize; the overall impression of the surface architecture of most 'hairy' cells suggests, however, that they are related to the monocytic series. From the examination of these cases it is evident that scanning electron microscopy may be used as a means of distinguishing chronic lymphocytic leukaemia from 'hairy' cell leukaemia on the basis of surface ultrastructure.
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PMID:'Hairy' cell leukaemia (leukaemic reticuloendotheliosis): a scanning electron microscopic study of eight cases. 119 57

In seven patients the diagnosis of hair cell leukaemia (leukaemic reticuloendotheliosis) was confirmed cytochemically and histologically. Splenectomy, in six patients, apparently favourably influenced the course. Isoenzyme 5 of the acid phosphatase was demonstrated in the hairy cells of all patients. Reaction of alpha-naphthylacetate esterase was moderately positive in the hairy cells. Phagocytosis of latex and India-ink particles was demonstrated especially in tartrate-resistant cells of one patient. In two patients eight permanently growing cell lines were demonstrated from leucocytes and defined cytochemically. Capacity for phagocytosis of hairy cells and positive reaction of alpha-naphthylacetate esterase in the hairy cells suggest properties of monocytes. But it is not possible definitively to classify the hairy cells among B-cells or monocytes.
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PMID:[Hairy cell leukaemia. I. Clinical features, cytochemistry, phagocytosis, establishment of permanent growing cell lines (author's transl)]. 124 50

Hairy cell leukemia (HCL) is an uncommon chronic lymphoproliferative disorder, its treatment requires continuous update. Splenectomy as first line therapy has few indications; recombinant alfa interferon (IFN) leads to a high overall response rate but there are few bone marrow remissions; deoxycoformycin (dCF) or pentostatin leads to a higher complete bone marrow response rate than with IFN but follow-up biopsies show persistence of hairy cells; 2-chlorodeoxyadenosine (2-CdA) is a purine analog that after a single seven day intravenous infusion leads to a complete response rate. 2-CDA will probably become the drug of choice for first line therapy for HCL.
Leukemia 1992 Nov
PMID:Hairy cell leukemia 1992. 127 27

Lymphadenopathy is an uncommon finding in hairy cell leukaemia (HCL). We report 12 HCL patients in whom relapse was associated with massive abdominal lymphadenopathy. All but one had long-standing HCL (range 3-25 years; median 10 years); in one it was discovered at presentation. Nine patients had been splenectomized and seven had previously been treated with 2'deoxycoformycin (DCF) and/or alpha-interferon (alpha IFN): three had achieved complete remission and four a partial response. The computerized tomography (CT) scan appearances were similar in all cases with a primary lymph node mass centred around the coeliac axis and involving upper para-aortic and retropancreatic regions. Histology and/or cytology confirmed nodal involvement by HCL in six patients. Large immature hairy cells were seen in both lymph nodes and bone marrow, suggesting a degree of transformation. Nine patients were treated with DCF: one had complete resolution, six responded with 50-90% reduction of the lymphadenopathy, one did not respond and one is still on treatment; alpha-IFN was used concomitantly or sequentially in two of the responders. One responding patient died of sepsis after four injections of DCF. Three patients received either alpha- or beta-IFN alone with no response. One elderly patient was not treated. Abdominal lymphadenopathy could be part of the natural history of HCL and/or may represent a transformation analogous to that seen in other low-grade lymphoproliferative disorders. Routine abdominal CT scanning should be part of the work up of all patients with HCL.
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PMID:Massive abdominal lymphadenopathy in hairy cell leukaemia: a report of 12 cases. 128 78

This study analyzes the association of Epstein-Barr virus (EBV) with non-Hodgkin's lymphoma (NHL) arising in patients without pre-existing overt immunodeficiency. The authors examined 201 lymphomas (105 high-grade B-cell, 82 peripheral T-cell, 7 high-grade non-B-cell, non-T-cell, and 7 hairy-cell leukemia) for EBV gene expression by immunohistologic procedures using monoclonal antibodies to EBV latent, immediate early, and replicative infection antigens. Transformation-associated EBV latent membrane protein 1 (LMP 1) was detected in 13 (6%) NHL, comprising 4 (4%) high-grade B-cell, 8 (10%) peripheral T-cell, and 1 non-B-cell, non-T-cell lymphomas. Anaplastic large-cell lymphoma of T-cell type was consistently LMP 1-negative. EBV nuclear antigen 2 was demonstrated in only three (1%) cases. Induction of replication as defined by expression of the immediate early BamHI Z leftward reading frame 1 (BZLF1) protein was detected in five cases, but early (EA) and late (VCA and MA) lytic cycle antigens were only found in two cases and in one case, respectively. The presence of EBV was confirmed by in situ DNA hybridization in 9 of 11 EBV antigen-positive lymphomas. This study shows the surprisingly frequent presence of EBV in peripheral T-cell NHL in European patients without pre-existing overt immunodeficiency. Interestingly, most sporadic B-cell NHL are not associated with the virus. Furthermore, the usefulness of selected monoclonal antibodies for the routine immunohistological diagnosis of EBV infection was confirmed.
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PMID:A survey of Epstein-Barr virus gene expression in sporadic non-Hodgkin's lymphomas. Detection of Epstein-Barr virus in a subset of peripheral T-cell lymphomas. 131 39

Since 1984 alpha-interferon (alpha IFN) has been the treatment of choice in cases of hairy-cell leukaemia. However, eight years' experience shows that, although a large majority of the patients are improved, they are not cured. Thus, a new type of cytostatic drug, perhaps capable of cure as well, may replace alpha-IFN for the treatment of hairy-cell leukaemia in the future.
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PMID:[Alpha interferon treatment in hairy cell leukemia]. 134 20

2-Chlorodeoxyadenosine (CdA) is active in chronic lymphocytic leukemia, hairy-cell leukemia, and low-grade lymphomas. In part, this spectrum of activity may be attributable to the selective toxicity of CdA to nondividing lymphocytes and monocytes. However, CdA is unstable at acidic pH and is degraded by bacterial nucleoside phosphorylases. The present experiments demonstrate that the 2'-arabino-fluoro derivative of CdA, designated CAFdA, is also directly toxic to quiescent lymphocytes and macrophages. Unlike CdA, CAFdA was stable at pH 2 and resisted degradation by Escherichia coli nucleoside phosphorylase. Cell killing was preceded by the formation of DNA strand breaks and could be prevented by supplementation of the medium with deoxycytidine. The initial DNA damage initiated the pattern of oligonucleosomal DNA fragmentation characteristic of apoptosis. Mutant lymphoblasts, deficient in deoxycytidine kinase, with elevated cytoplasmic 5'-nucleotidase, or with expanded deoxynucleotide pools secondary to increased ribonucleotide reductase activity, were cross-resistant to both CAFdA and CdA toxicity. One-week oral treatment with CAFdA (1 mg/ml in drinking water) achieved an average plasma concentration of 0.56 microM and eliminated 90% of chronic lymphocytic leukemia cells transplanted into severe combined immunodeficiency (scid) mice. Under the same conditions, CdA was much less active. Collectively, these results suggest that CAFdA could be effective as an oral agent in indolent lymphoproliferative diseases and in autoimmune diseases where lymphocyte and monocyte depletion is desirable.
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PMID:Oral antilymphocyte activity and induction of apoptosis by 2-chloro-2'-arabino-fluoro-2'-deoxyadenosine. 134 62

The treatment of hairy cell leukemia (HCL) requires continuing update. The role for splenectomy is extremely limited. Alfa-interferon therapy has been available and utilized for five years; there have been few complete remissions. The experience with Pentostatin has resulted in a majority of clinical complete remissions after several months of therapy, but persistence of a small percentage of hairy cells in the bone marrow. The most recent therapeutic agent, 2-chloro-deoxyadenosine (2-CDA) is given for seven days and results in a complete remission in the great majority of patients with no evidence of persistent bone marrow disease. If this trend persists, 2-CDA will become first line therapy for HCL as it may cure the disease.
Leukemia 1992
PMID:The treatment of hairy cell leukemia: an update. 134 62

Several new antimetabolites have been evaluated in clinical trials in recent years. Those with the most promising activity include the structurally related purine analogs fludarabine, 2-chlorodeoxyadenosine, and 2'-deoxycoformycin. These compounds have shown impressive activity against a broad spectrum of indolent lymphoproliferative disorders, including hairy-cell leukemia, chronic lymphocytic leukemia, and low-grade non-Hodgkin's lymphomas. They may also be useful in the treatment of acute leukemias. In contrast, they lack activity against common solid tumors. They have been generally well tolerated in large clinical trials; however, each of them is myelosuppressive and immunosuppressive. It is unlikely that any one of these drugs, when used as a single agent, will provide optimal therapy for any disease other than, possibly, hairy-cell leukemia. Combinations with other cytotoxic agents and biologics are in development, and perhaps they will lead to more effective regimens in the future.
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PMID:New antimetabolites in the treatment of human malignancies. 136 Oct 80


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