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Hairy-Cell Leukaemia

  • Kartonierter Einband
  • 140 Seiten
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Hairy-cell leukaemia (HCL) is an established clinicopathological entity typified by a chronic picture of substantial splenomegaly,... Weiterlesen
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Hairy-cell leukaemia (HCL) is an established clinicopathological entity typified by a chronic picture of substantial splenomegaly, less frequent hepatomegaly, and only inconspicuous lymphadenopathy. Central to the diagnosis is the pathognomonic hairy cell (HC), a dis tinctive mononuclear cell that circulates in varying numbers and infiltrates a variety of organs, including the bone marrow and spleen. Peripheral pancytopenia is often present, and circulating HCs may be infrequent. HCs are invariably present in the bone marrow; but, per haps because of the extensive fibrosis characteristic of the disease, the marrow is frequently difficult to aspirate, and confirmation of the diagnosis may require histopathological ex amination of biopsy material. The incidence of the disease has been given as 2% ofleukaemias [29, 319] or as approximately 1 % of identifiable lymphomas [230]. Our own experience of collecting cases in the United Kingdom leads us to expect that at any given time one patient with HCL will be attending a clinic serving some 150,000 people.

1 Introduction.- 2 Clinical Aspects.- 2.1 Occurrence.- 2.2 Clinical Features at Presentation.- 2.2.1 Age and Sex Distribution.- 2.2.2 Symptoms and Signs.- 2.3 Haematology at Presentation.- 2.3.1 Peripheral Blood.- 2.3.2 Bone Marrow Aspiration.- 2.4 Other Investigations.- 2.4.1 Blood Chemistry.- 2.4.2 Serum Proteins.- 2.4.3 Erythrocyte Sedimentation Rate (ESR).- 2.4.4 Radiology.- 2.5 Treatment.- 2.5.1 Splenectomy.- 2.5.2 Chemotherapy.- 2.5.3 Irradiation.- 2.6 Clinical and Haematological Progress.- 2.6.1 Survival and Prognostic Features.- 2.6.2 Course and Fluctuations in the Disease.- 2.7 Complications and Cause of Death.- 2.7.1 Infections.- 2.7.2 Noninfectious Complications.- 3 Pathology.- 3.1 Bone Marrow.- 3.2 Spleen.- 3.3 Liver.- 3.4 Lymph Nodes.- 4 The Hairy Cell: Cytological Aspects.- 4.1 Cytology.- 4.2 Cytochemistry.- 4.2.1 Acid Phosphatase.- 4.2.2 Esterase.- 4.2.3 Periodic Acid-Schiff (PAS).- 4.2.4 ?-Glucuronidase.- 4.2.5 Other Cytochemical Methods.- 4.2.6 Terminal Deoxynucleotidyl Transferase (TdT).- 4.3 Ultrastructure.- 4.3.1 Transmission Electron Microscopy (TEM).- 4.3.2 Scanning Electron Microscopy (SEM).- 4.4 Cytokinetics.- 4.5 Cytogenetics.- 4.6 Metabolism.- 4.7 Culture.- 5 The Hairy Cell: Immunological Aspects.- 5.1 Transformation and Cytotoxicity Studies.- 5.1.1 Mitogen-Induced Transformation.- 5.1.2 Mixed Lymphocyte Reactions (MLR).- 5.1.3 Cytotoxicity Functions.- 5.2 Surface Receptors.- 5.2.1 Sheep Erythrocyte (E) Rosettes and Anti-T Cell Sera.- 5.2.2 Surface Immunoglobulin (Sig).- 5.2.3 Fc Receptors.- 5.2.4 Receptors for Fixed C3 and the Epstein-Barr Virus.- 5.2.5 Receptors for Mouse Erythrocytes.- 5.2.6 Other Markers.- 5.3 Monocytic Features.- 5.3.1 Morphological Aspects.- 5.3.2 Adhesive Properties and Phagocytosis.- 5.3.3 ?Fc Receptors and the la Antigen.- 5.3.4 Monocytopenia and Reticuloendothelial Distribution.- 5.4 Immunoglobulin Synthesis and Secretion.- 5.4.1 Immunoglobulin Secretion.- 5.4.2 Cytoplasmic Immunoglobulin.- 5.4.3 Paraproteinaemia.- 5.5 Summary of the Immunological Membrane Phenotype of Typical HCs.- 5.6 T-cell Features.- 6 Other Haemic Cells.- 6.1 Granulocytes.- 6.1.1 Neutrophils.- 6.1.2 Eosinophils and Basophils.- 6.2 Monocytes.- 6.3 Platelets.- 6.4 Lymphocytes.- 6.4.1 T Cells.- 6.4.2 B Cells.- 7 Diagnosis.- 7.1 Differential Diagnosis.- 7.2 A New Distinct Entity.- 8 Conclusions and Future Trends.- 9 References.- 10 Subject Index.


Titel: Hairy-Cell Leukaemia
EAN: 9783642814396
ISBN: 3642814395
Format: Kartonierter Einband
Herausgeber: Springer Berlin Heidelberg
Genre: Medizin
Anzahl Seiten: 140
Gewicht: 256g
Größe: H244mm x B170mm x T7mm
Jahr: 2011
Auflage: Softcover reprint of the original 1st ed. 1980

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