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

Over a 4-year period 203 patients with various types of leukaemia were treated by the Haematology Unit at the Johannesburg Hospital. Ten of them were suffering from the condition known as hairy-cell leukaemia or leukaemic reticulo-endotheliosis. They were all men, and ranged in age from 29 to 67 years (mean 56 years). The majority presented with pancytopenia, and there was invariably splenomegaly, while lymphadenopathy was rare. Hairy cells were identified microscopically in the peripheral blood of 7 patients and in 5 the specific cytochemical marker, tartrate-resistant acid phosphatase, was present. In addition, in a further 2 patients this feature, which was not identified in the peripheral blood, was found in the splenic cells. The bone marrow trephine biopsy specimens characteristically showed extensive lymphoid infiltration associated with a dense disordered deposition of reticulin fibres. Electron microscopical and immunological studies proved to be of doubtful diagnostic value. Splenectomy was carried out on 9 patients, and there was tumour involvement in all the spleens removed. Two patients died from septicaemia, the one before splenectomy and the other 9 months after the operation. The 8 remaining patients have had their subjective symptoms alleviated and their peripheral blood indices have been improved by splenectomy, and none has required further treatment for periods now ranging from 7 to 41 months.
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PMID:The diagnosis and management of hairy-cell leukaemia. 8 70

Cytochemical and immunological studies were performed on "hairy" cells from 5 patients. The results, primarily the detection of surface immunoglobulins after incubation in immunoglobulin-free medium support the hypothesis that hairy cells are B-cells. Further parameters for B-cell lineage are the failing suppression of unspecific esterases by NaF, the presence of tartrate-resistant acid phosphatase and the absence of phagocytic capacity and glass adherence (skin window). A lysozyme could not be demonstrated. Of two patients with prolymphocytic leukemia one was characterized as T- and one as B-cell leukemia. Therefore, the prolymphocytic leukemia has to be discussed as intermediate form of ALL and CLL. In both patients the therapeutic results were discouraging with cytostatics (e. g. chlorambucil), splenectomy is considered as the treatment of choice.
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PMID:[Prolymphocytic and hairy cell leukemias as special forms of chronic lymphatic leukemia]. 29 12

In vitro studies were performed with leukaemic cells from two patients with hairy cell leukaemia in order to define the nature and kinetics of immunoglobulin synthesis by the neoplastic cells. Both patients had clinically and morphologically well-defined disease and their cells contained abundant tartrate-resistant acid phosphatase. One patient had associated macroglobulinaemia. The hairy cells had B-lymphocyte characteristics as determined by fluorescent immunoglobulin staining and surface receptor properties. They synthesized monoclonal IgM and IgG respectively in vitro. The kinetics of immunoglobulin synthesis were different in cells from the two patients as measured by equilibration time, intracellular degradation, and secretion. Permanent cell lines were established with cells from these patients. The lines grow as typical B-lymphoblastoid cultures and continue to produce tartrate-resistant acid phosphatase and immunoglobulin. These studies unequivocally demonstrate the B-lymphocyte nature of the hairy cells in these patients and provide evidence for their clonal origin both in terms of immunoglobulin and enzyme synthesis.
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PMID:Immunoglobulin synthesis in hairy cell leukaemia. 85 55

Differential characterization of the "reticulum cell" in lymphoreticular neoplasms. Am J Clin Pathol. 64: 171-179, 1975. The term "reticulum cell" is confusing, having been applied to the cells involved in many hematopoietic neoplasms, such as reticulum-cell sarcoma, histiocytic medullary reticulosis, leukemic reticuloendotheliosis, and monocytic or histiocytic leukemias. In histologic sections, even the cells from poorly differentiated extramedullary lesions of chloroma or myeloblastic leukemia have been called "reticulum cells."A combined morphologic and cytochemical approach has been used to study "reticulum cells"in smears and tissue sections of neoplasms involving "histiocytes" or "reticulum cells."The cytochemical markers are: chloracetate esterase for neutrophilic granulocytes; nonspecific esterase and fluoride-resistant esterase for monocytes and histiocytes (phagocytes); tartrate-resistant acid phosphatase for the reticulum cells of leukemic reticuloendotheliosis; pyronin for the lymphatic reticulum cells (germinal center cells). The morphology of these cells is very well appreciated in smears, and the locations of these marked cells in tissue sections are easily recognized. The use of cytochemical and immunochemical methods and functional studies, in addition to simple morphology, may be useful in subclassification of lymphoreticular neoplasms.
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PMID:Differential characterization of the "reticulum cell" in lymphoreticular neoplasms. 109 40

Most of the circulating lymphocytes from three asymptomatic adults (one male, two female, age range 61-67 years) with isolated persistent lymphocytosis of between 7.1 and 10 x 10(9)/l possessed characteristic villous projections of the cell membrane. Morphological, histochemical, ultrastructural, immunological, and genotypic studies confirmed a clonal proliferation of tartrate-resistant acid phosphatase (TRAP)-negative CD5-CD10-CD25- and CD11c+ B-cells. In addition to CD11c, these cells expressed other adhesion receptors (LFA-1/CD11a, VLA-4/CD29/49d, ICAM-1/CD54, and LAM-1) and produced detectable amounts of interleukin-1 beta, interleukin-6, and in one case tumour necrosis factor-alpha mRNA. This monoclonal villous lymphocytosis (MVL) could be differentiated from B-cell chronic lymphocytic, prolymphocytic, and hairy cell leukaemias, and from previously recognized CD11c+ chronic B-cell leukaemia. A rare splenomegalic non-Hodgkin's lymphoma variant with circulating villous B-lymphocytes (SLVL), usually CD10+ and sometimes CD11c- and TRAP+, appears to be a closely related disorder. In all three patients the lymphocyte count increased very slowly, at a rate less than 5 x 10(9)/l per year, over 3-7.5 years of follow up, and a moderate splenomegaly eventually developed in one of the patients. Chemotherapy was never required. MVL may be a relatively benign clinical entity akin to SLVL within the group of CD11c+ B-cell lymphoproliferative disorders.
Leukemia 1991 Sep
PMID:Monoclonal lymphocytosis with villous lymphocytes: a chronic lymphoproliferative disease of CD11c+ B-cells. 168 36

The cardinal features of hairy cell leukaemia are: (i) cytopenias, (ii) splenomegaly, and (iii) mononuclear cells of B-cell origin with cytoplasmic projections and tartrate-resistant acid phosphatase-positivity. The most common complication is infection. In the past, the mainstay of therapy has been splenectomy, and this procedure is still often suggested as a first-line approach. However, research during the last decade has resulted in three new, highly effective therapies for hairy cell leukaemia: interferon-alpha (IFN-alpha), 2'-deoxycoformycin (DCF) and 2-chlorodeoxyadenosine (2CDA). IFN-alpha is currently approved for this indication. About 90% of patients have a durable haematologic recovery, and complete remission rates range from less than 5% to greater than 40% in different series. It should be noted that patients with partial remissions generally have normal or near-normal blood counts, and can live indefinitely without disease-related problems, despite a few remaining hairy cells in the bone marrow. In this paper we will discuss the various therapeutic modalities available for patients with hairy cell leukaemia.
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PMID:Hairy cell leukaemia: review of treatment. 171 89

Hairy-cell leukemia is a lymphoid leukemia of B-cell lineage, the cells of which are characteristically tartrate resistant acid phosphatase positive on blood and bone marrow smears. However, because hairy-cell leukemia is frequently associated with abundant marrow stroma, dry marrow taps, and peripheral pancytopenia, the diagnosis may rest on the appearance of the bone marrow biopsy specimen alone. The ribosome-lamella complex has been associated with hairy-cell leukemia, and can be visualized by light microscopy using l-micron sections of plastic-embedded bone marrow specimens stained with toluidine blue. We describe the findings in a case in which bone marrow and liver biopsy specimens were positive for hairy cells containing ribosome-lamella complex, which were visualized with both electron microscopy and light microscopy. Reliable light microscopic identification of ribosome-lamella complex may provide an easy and inexpensive method of aiding in the diagnosis of hairy-cell leukemia when aspirate material is not available for tartrate-resistant acid phosphatase stain.
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PMID:Visualization of the ribosome-lamella complex in plastic-embedded biopsy specimens as an aid to diagnosis of hairy-cell leukemia. 176 18

In order to investigate the possible mechanisms for the effect of alpha-interferon (alpha-IFN) in hairy cell leukaemia (HCL), blood cells from 4 cases were treated in vitro with alpha-IFN, tumour necrosis factor (TNF) and interleukin 2 (IL-2). Changes in the antigen expression, immunoglobulin (Ig) secretion and the production of TNF and lymphotoxin (LT) were investigated. TNF induced expression of CD4 and CD71, increased the intensity of HLA-DR, CD25, CD11c and CD13 expression and decreased both the intensity and frequency of sIg and cIg positivity. alpha-IFN decreased CD25 expression, the tartrate-resistant acid phosphatase activity (TRAP), reduced the TNF-induced CD4 and CD71 expression and antagonized the TNF effect on the Ig expression. Spontaneous TNF or LT production could not be detected in culture supernatants. However, TNF was found to induce LT production, an effect which alpha-IFN antagonized and IL-2 augmented. The reduction of CD25, TNF-induced CD71 and TRAP caused by alpha-IFN seems to represent a deactivation of the activated state of hairy cells (HCs). The failure of alpha-IFN to induce Ig secretion or CD38 expression in HCs speaks against a differentiation induction effect. The LT secretion induced by TNF suggests that other cytokines than TNF might be involved in the proliferation of HCs and that alpha-IFN by blocking the production of LT and perhaps other cytokines causes a growth arrest in HCs.
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PMID:Effect of alpha-IFN on cytokine-induced antigen expression and secretion of TNF, LT and IgM in HCL. 192 51

Neoplastic lymphocytes with a hairy appearance were detected in the ascitic fluid from a case of retroperitoneal malignant lymphoma. Although the tumor cells resembled those of hairy-cell leukemia (HCL), no leukemic change was observed, and the anatomic location of the neoplastic cells was different from that seen in HCL. The tumor cells were positive for some immunohistochemical markers of HCL (i.e., CD1 9 and SIg) but were negative for others (CD11c, CD25 and tartrate-resistant acid phosphatase). Immunocytofluorometric and postmortem histologic studies showed the lesion to be a well-differentiated B-cell lymphocytic lymphoma with plasmocytic differentiation in some cells.
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PMID:Cytologic detection of malignant lymphoma cells with a hairy appearance in ascitic fluid. Report of a case with immunofluorocytometric analysis. 192 92

We observed a case of hairy cell leukaemia without splenomegaly and without hairy cells in blood that was difficult to diagnose. Typical hairy cells with cytoplasmic projections were found in hypocellular bone marrow smears rarely whereas atypical mononuclear cells of hairy cell type constituted 70% and 1.5% of them exhibited ring-shaped nuclei. These cells were in 8% positive for tartrate-resistant acid phosphatase. Bone marrow biopsy revealed diffuse infiltration with hairy cells. We reviewed bone marrow smears of 11 other patients with hairy cell leukaemia and we found hairy cells with ring-shaped nuclei in one of them only with a frequency of one promile.
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PMID:[A diagnostically difficult case of hairy cell leukemia with ring-shaped nuclei]. 204 40


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