1 Introduction and Historical Aspects.- 2 Clinical Aspects.- 2.1 Clinical features.- 2.1.1 Epidemiology.- 2.1.2 Clinical features.- 2.2 Laboratory features.- 2.2.1 Haematology.- 2.2.2 Other clinical laboratory investigations.- 2.3 Prognosis: staging systems.- 3 Pathology.- 3.1 Bone marrow.- 3.2 Spleen.- 3.3 Liver.- 3.4 Lymph nodes.- 3.5 Other blood cells.- 3.5.1 Monocytes.- 3.5.2 T cells.- 3.5.3 Other haemic cells.- 3.6 Viruses and HCL.- 4 Diagnosis and Hairy-like Disorders.- 4.1 The diagnosis of typical HCL.- 4.2 Diagnosis when few abnormal cells are present.- 4.3 Diagnosis of HCL in the presence of atypical clinical or pathological features.- 4.4 Variant forms of HCL and closely related conditions.- 4.5 Atypical and rare presentations.- 5 Treatment: Mechanisms.- 5.1 Mechanism of action of splenectomy.- 5.2 The mechanism of action of ?IFN.- 5.2.1 Indirect effects.- 5.2.2 'Direct' anti-proliferative activity.- 5.2.3 Modification of intracellular signals by ?IFN.- 5.2.4 Altered differentiation/activation state.- 5.3 Mechanism of action of the purine analogue drugs.- 5.3.1 Normal purine metabolism in lymphoid cells: the role of adenosine deaminase.- 5.3.2 Adenosine deaminase function in the presence of purine analogue drugs.- 5.3.3 Action of purine analogue drugs on malignant cells.- 6 Treatment: Practical Considerations.- 6.1 When should treatment be commenced?.- 6.2 Splenectomy.- 6.2.1 Therapeutic value of splenectomy.- 6.3 Interferon.- 6.3.1 Therapeutic activity of IFN in HCL.- 6.3.2 Problems with IFN therapy.- 6.3.3 Alternative strategies.- 6.4 Purine analogue drugs: DCF, CDA and fludarabine.- 6.4.1 Therapeutic activity of DCF.- 6.4.2 Therapeutic activity of CDA.- 6.4.3 Fludarabine.- 6.4.4 Choice of purine analogue drug in HCL.- 6.5 The use of colony-stimulating factors in HCL.- 6.6 Overview of therapeutic options.- 6.7 Treatment after failure of primary treatment option.- 6.8 Other treatment options.- 7 The Hairy Cell.- 7.1 Cytology.- 7.2 Cytochemistry.- 7.2.1 Tartrate-resistant acid phosphatase (TRAP).- 7.2.2 Other cytochemical techniques.- 7.3 Cytogenetics.- 7.3.1 Frequency of karyotypic abnormalities.- 7.3.2 Specificity of chromosomal changes.- 7.3.3 Possible significance of karyotypic changes in HCL.- 7.4 Ultrastructure.- 7.4.1 Transmission electron microscopy.- 7.4.2 Scanning electron microscopy.- 7.5 Nature of the malignant cell.- 7.5.1 Developmental stage: evidence from immunoglobulin studies.- 7.5.2 Developmental stage: evidence from immunophenotypic studies.- 7.5.3 Developmental stage: other approaches.- 7.6 Membrane, cytoskeletal organisation and signalling.- 7.6.1 Membrane.- 7.6.2 Cytoskeleton.- 7.6.3 Signalling.- 7.7 Response to cytokines.- 7.7.1 Low molecular weight B-cell growth factor.- 7.7.2 Tumour necrosis factor.- 7.7.3 Interleukin-2.- 7.7.4 Other interleukins.- 7.7.5 Myeloid growth factors.- 7.8 Adhesion and tissue localisation.- 7.8.1 Non-integrin adhesion receptors.- 7.8.2 Integrins.- 7.8.3 Adhesion molecules and HC behaviour.- 7.9 Cell survival and cell death.- Appendices.- Appendix A: Tartrate-resistant acid phosphatase (TRAP).- Appendix B: CD-antigen expression by HCs.- References.
Hairy cell Leukaemia (HCL) has always attracted an interest out of all proportion to its frequency and continues to do so. There are two reasons for this. The first is that the disease is unusually responsive to therapy and second is that it has provided a number of important insights into B-cell biology. This monograph is a comprehensive account of hairy cell leukaemia and aims to provide a more detailed account than is available in the existing literature. The work is timely because a consensus has now emerged concerning accurate differential diagnosis a nd curative treatment. These aspects therefore form the focus of the book and are considered in detail. The basic advances in the laboratory that encourage the belief that elucidation of the underlying oncogenic event in the disease may be within reach. The background to this belief is extensively renewed. As a result the monograph will be of interest and practical value to both clinicians and researchers in this and related fields.
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