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Book Cover
Author Schrezenmeier, H. (Hubert)

Title Aplastic Anemia : Pathophysiology and Treatment
Published Cambridge, UK ; New York, NY ; Cambridge, UK : Cambridge University Press, 2000
Cambridge : Cambridge University Press, 1999
Online access available from:
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Description 1 online resource (406 pages)
Contents Cover -- Half-title -- Title -- Copyright -- Contents -- Contributors -- Preface -- Part I Pathophysiology of acquired aplastic anemia -- 1 Stem cell defect in aplastic anemia -- Abbreviations -- Normal stem cells -- Definition -- Regulation -- The experimental study of primitive hemopoietic cells -- Selection of primitive cells by phenotype -- How many stem cells get to express themselves? -- Aplastic anemia stem cells -- Functional assessment of AA hemopoietic progenitor cells -- Phenotypic quantitation of AA hemopoietic (CD34+) cells -- Assessment of the long-term marrow-repopulating ability of AA hemopoietic cells -- Mobilizing potential of AA progenitor cells -- Apoptotic properties of AA CD34+ cells -- Analysis of telomeric DNA length in AA -- Conclusion -- References -- 2 Cytokine abnormalities in aplastic anemia -- Introduction -- Erythropoietin (EPO) -- Granulocyte colony-stimulating factor (G-CSF) -- Thrombopoietin (TPO) -- Stem cell factor (SCF) -- Granulocyte-macrophage colony-stimulating factor (GM-CSF) -- Flt-3 ligand (Flt-3L) -- Interleukin-1 (IL-1) -- Production of cytokines by marrow stromal cells -- Rationale for the therapeutic use of cytokines -- Conclusion -- References -- 3 Role of T-lymphocytes in the pathophysiology of aplastic anemia -- Introduction -- Immunophenotyping data on T-cells and natural killer cells in bone marrow and peripheral blood -- CD4 and CD8 T-cells -- Activated T-cell subpopulation -- T-cell receptor VBeta subfamily -- Natural killer cells -- Markers of T-cell activation -- Activation antigens -- Cytokine production -- Stress proteins -- Production of myelosuppressive cytokines by T-lymphocytes -- Reactivity of T-lymphocytes against hemopoietic progenitor cells -- Evidence of the involvement of an antigen-driven response in the development of AA
Hemopoietic progenitor cells as a target of T-cell attack -- Association of certain HLA alleles with a susceptibility to AA -- Clonal predominance in T-cell subsets defined by T-cell receptor VBeta expression -- Establishment of a T-cell clone reactive to hemopoietic cells from AA patients -- Conclusion -- References -- 4 Role of apoptosis in the pathophysiology of aplastic anemia -- Introduction -- Features of apoptotic cells -- Control of apoptosis -- Apoptosis: role in immune regulation -- Methods of detecting apoptotic cells -- Apoptosis in progenitor cells in the bone marrow of those with aplastic anemia -- Expression of Fas on normal hemopoietic progenitor cells -- Expression of Fas on progenitor cells in aplastic anemia -- Modulation of apoptosis by therapeutic intervention -- Conclusion -- References -- 5 The interrelation between aplastic anemia and paroxysmal nocturnal hemoglobinuria -- Introduction -- AA-PNH syndrome - clinical observations -- The deficiency of GPI-anchored proteins in AA-PNH syndrome -- The GPI anchor and its biosynthesis (reviewed in Rosse, 1997 -- Socié, 1997) -- The most widely studied GPI-anchored proteins (reviewed in Rosse, 1997 -- Socié, 1997) -- The GPI-anchored protein deficiency (reviewed in Rosse, 1997 -- Socié, 1997) -- The GPI-anchored protein deficiency in AA-PNH syndrome -- Mutations of the PIG-A gene in AA-PNH syndrome -- Molecular biology of the PIG-A gene -- Abnormalities of the PIG-A gene in PNH -- Abnormalities of the PIG-A gene in AA-PNH syndrome -- Conclusion: the mutations of the PIG-A gene as a common genetic basis for PNH and AA-PNH syndrome -- References -- 6 Aplastic anemia and other clonal disorders -- Introduction -- Clinical observations: the evolution of AA to myelodysplastic syndrome and acute myelogenous leukemia
Studies on clonal hemopoiesis in AA patients using X-linked DNA polymorphisms -- Cytogenetic abnormalities in patients with severe aplastic anemia -- Conclusion -- References -- Part II Epidemiology and clinical features of acquired aplastic anemia -- 7 Epidemiology and etiology of aplastic anemia -- Introduction -- The role of medical drugs: a good old theory revisited -- Environmental and industrial chemicals -- Viruses -- Risk factors others than exposure -- Conclusion -- References -- 8 Clinical presentation, natural course, and prognostic factors -- Clinical manifestations -- Complementary investigations -- Peripheral blood cell counts -- Other analytical data -- The cytology and histopathology of medullary aplasia -- Isotopic investigations of hemopoiesis -- Proton magnetic resonance imaging and spectroscopy -- Follow-up and survival -- Prognosis identification -- The prognostic value of symptoms -- The prognostic value of peripheral blood cell counts -- The prognostic value of the cytology and histopathology of medullary aplasia -- The prognostic value of isotope examinations -- Statistical prediction models -- International criteria -- Conclusion -- References -- Part III Treatment of acquired aplastic anemia -- 9 Supportive treatment of patients with severe aplastic anemia -- Introduction -- Prevention and treatment of infections -- Risk of infections -- Types of infections in SAA patients -- Prevention of infections -- Antibacterial chemoprophylaxis -- Antiviral prophylaxis -- Diagnosis of infection -- Investigation of fever -- Investigation of patients with pulmonary infiltrates -- Treatment of infection -- Treatment of bacterial infections -- Treatment of fungal infections -- Treatment of viral infections -- Transfusion support -- Transfusions and risk for graft rejection after BMT -- Alloimmunization after transfusion
Transmission of infectious agents -- Transfusion-associated GVHD -- Iron overload -- Conclusion -- References -- 10 Immunosuppressive treatment of aplastic anemia -- Introduction -- Lessons from the clinical trials -- Effectiveness of ALG -- The impact of severity of disease -- Cyclosporin -- Intensification of immunosuppression -- Adjuncts to immunosuppression -- Splenectomy -- Timing and quality of response -- Mechanism of action of ALG and cyclosporin -- Treatment schedules -- Adverse events of ALG -- Immunosuppression in elderly patients -- Relapses and nonresponses -- New approaches to immunosuppressive treatment -- Conclusion -- Acknowledgements -- References -- 11 Role of cytokines in the treatment of aplastic anemia -- The rationale and potential aims of growth factor treatment in aplastic anemia -- Serum/plasma levels and production of endogenous growth factors in aplastic anemia -- Clinical trials of hemopoietic growth factors in aplastic anemia -- Granulocyte colony-stimulating factor -- Granulocyte/macrophage colony-stimulating factor (GM-CSF) -- Erythropoietin -- Interleukin-3 (IL-3) -- Interleukin-1 (IL-1) -- Interleukin-6 (IL-6) -- Stem-cell factor (SCF) -- Conclusion -- References -- 12 HLA-identical sibling bone marrow transplantation to treat severe aplastic anemia -- Introduction -- HLA-identical sibling transplants -- Survival, current results, and trends over time -- Engraftment -- Hemopoietic chimerism -- Acute GVHD -- Chronic GVHD -- Interstitial pneumonitis -- Late complications -- The influence of SAA etiology on outcome -- Syngeneic transplants -- HLA-phenotypically identical donors -- Conclusion -- References -- 13 Alternative donor bone marrow transplantation for severe acquired aplastic anemia -- Introduction -- Prognostic factors for HLA-identical sibling BMT -- Partially matched family donor BMT: early experience
HLA-matched unrelated donor BMT: early experience -- Analysis of factors affecting outcome of alternative donor BMT -- Patients and methods -- Patient population -- Donor population and HLA matching -- Transplant data -- Outcomes analyzed -- Statistical methods -- Results -- Survival -- Engraftment -- Acute GVHD -- Chronic GVHD -- Cause of death -- Discussion -- Future directions -- Transfusion policy in patients with SAA -- HLA molecular epitope analysis -- Conclusion -- References -- 14 Treatment of children with acquired aplastic anemia -- Introduction -- BMT from HLA-identical siblings -- BMT from alternative donors -- Immunosuppressive treatment -- Hemopoietic growth factors -- Late effects on children -- Treatment results obtained in the last 5 years in Europe -- Conclusion -- References -- 15 Long-term follow-up of patients with aplastic anemia: clonal malignant and nonmalignant complications -- Introduction -- Historical background -- Secondary MDS or leukemia -- New solid cancers -- Paroxysmal nocturnal hemoglobinuria -- Conclusion -- References -- 16 Guidelines for treating aplastic anemia -- Decision making: immunosuppressive treatment versus allogeneic bone marrow transplantation as first-line treatment of… -- Bone marrow transplantation -- HLA-matched sibling transplantation -- Unrelated and family-mismatched donor transplants -- Immunosuppressive treatment -- Failures to respond to first-line treatment/relapse -- Failures to respond to second-line treatment -- Age limit for immunosuppressive treatment -- Myelodysplastic syndrome/leukemia after treatment -- Conclusion -- Part IV Fanconi's anemia -- 17 Clinical features and diagnosis of Fanconi's anemia -- Clinical suspicion of Fanconi's anemia -- Diagnosis of FA -- Hematological manifestations -- Leukemia -- Myelodysplastic syndrome -- Cancer -- Liver disease -- Reproduction -- Prognosis
Summary Comprehensive and up-to-date clinical reference, with an emphasis on treatment
Notes Description based upon print version of record
ProQuest Ebook Central Rental
Bibliography Includes bibliographical references and index
Notes Description based on publisher supplied metadata and other sources
Subject Aplastic anemia
Bone marrow -- Diseases
Anemia, Aplastic--physiopathology
Form Electronic book
Author Bacigalupo, A
ProQuest (Firm)
LC no. 99010964
ISBN 9780511149047 electronic bk
OTHER TI ProQuest Ebook Central