| Description |
1 online resource |
| Contents |
Preface; References; Contents; Contributors; Part I: Sickle Cell Disease; Chapter 1: Clinical Manifestations of Sickle Cell Disease Across the Lifespan; Introduction; Pathophysiology, Epidemiology, and Disease Modulators; Pathophysiology; Epidemiology; Genetic Modulators of Disease Severity; Prognosis; Treatment; Systemic Complications; Anemia; Painful Crises; Infections; Fever and Bacteremia; Infection Prevention; Specific Infections; Central Nervous System; Cardiovascular; Pulmonary; Renal; The Hepatobiliary and Gastrointestinal Systems; Bone Involvement |
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Ocular Manifestations Leg Ulcers; Thrombosis; Priapism; Treatment Toxicity; Other Major Life Events Complicated by SCD; Clinical Challenges; Newborn Screening: Identifying Patients for Early Clinical Care; Risk Stratification; Stigma and the Patient Experience; Knowledge Gaps; References; Chapter 2: Pathobiology of Sickle Cell Disease Vaso-occlusion and Targeted Therapies; Adhesion Pathways; Adhesive Interactions of Red Cells and Leukocytes with the Endothelium; Neutrophil-RBC Interactions; Therapeutic Interventions Targeting Adhesion Molecules; Inflammatory Pathways |
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Adenosine and Invariant Natural Killer T (iNKT) Cells Leukotrienes; Oxidative Stress and Impaired Nitric Oxide Biology; Role of Fetal Hemoglobin; Anti-sickling Agents; Chronic Pain in Sickle Cell Disease; Role of Activated Coagulation in SCD; Targeting Coagulation in SCD; Heparin; Direct Thrombin and Factor X Inhibitors; Vitamin K Antagonists; Role of Platelets in SCD; Antiplatelet Agents; Summary; References; Chapter 3: Current Non-HSCT Treatments for SCD; Disease-Modifying Therapies; Hydroxyurea; Blood Transfusions; Hematopoietic Stem Cell Transplantation |
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Supportive Therapies Analgesics; Non-opioid Analgesics; Opioid Analgesics; Other Adjuvant Therapies for Pain; Antibiotics and Immunizations; Penicillin Prophylaxis; Immunizations; Fluids; Evolving Therapies; Drugs Targeting Adhesion; GMI-1070; Crizanlizumab (SelG1); Drugs Targeting Hemoglobin Polymerization; GBT440; Other Mechanism-Based Therapies Under Investigation; References; Chapter 4: Risk-Based Therapies for Sickle Cell Disease; Introduction; Importance of Severity Prediction for Children with Sickle Cell Disease; Disease Severity Predictor or Association? |
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Historical Attempts to Identify Severity Predictors Risk Prediction in the Modern Era; Risk Stratification When Universal Newborn Screening, Penicillin Prophylaxis, and Routine TCD Screening Utilization Were Inconsistent or Unknown; Summary of the Predictive Value of the Three Most Commonly Studied Predictive Variables; HbF as a Predictor of SCA Complications; Alpha Globin Gene Number as a Predictor of SCA-Related Complications; Reticulocyte Count as a Predictor of SCA Severity; Where Do We Go from Here?; Conclusions; References |
| Summary |
This book provides a comprehensive, state-of-the art review of hematopoietic stem cell transplantation (HSCT) for sickle cell disease (SCD). The book reviews new data about risk prediction for severe SCD, outlines the unique challenges of HSCT for patients with SCD, profiles the supportive care guidelines for patients who are undergoing HSCT, highlights our current understanding of the best transfusion support for SCD patients prior to, during and after HSCT, and provides new perspectives about the ethics of HSCT for pediatric patients with SCD. Published in the last few years, several landmark phase III trials that utilize matched unrelated and haploidentical donors for HSCT in SCD patients are also placed in context with respect to current management. Written by experts in the field, Sickle Cell Disease and Hematopoietic Stem Cell Transplantation is a valuable resource for physicians and researchers dealing with and interested in this challenging, yet exciting, curative therapy for sickle cell disease, that will help guide patient management and stimulate investigative efforts |
| Bibliography |
Includes bibliographical references and index |
| Notes |
Print version record |
| Subject |
Sickle cell anemia -- Treatment
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Hematopoietic stem cells -- Transplantation
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Haematology.
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HEALTH & FITNESS -- Diseases -- General.
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MEDICAL -- Clinical Medicine.
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MEDICAL -- Diseases.
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MEDICAL -- Evidence-Based Medicine.
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MEDICAL -- Internal Medicine.
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Hematopoietic stem cells -- Transplantation
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Sickle cell anemia -- Treatment
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| Form |
Electronic book
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| Author |
Meier, Emily R., editor
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Abraham, Allistair, editor
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Fasano, Ross M, editor
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| ISBN |
9783319623283 |
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3319623281 |
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