This volume reviews the fundamental understanding of this potentially life-threatening disease and the advances in treatment that have been achieved with the use of the monoclonal antibody eculizumab. Although the PIGA gene has been known for many years, the mechanism of clonal dominance in paroxysmal nocturnal hemoglobinuria is still largely unknown. This book, Paroxysmal Nocturnal Hemoglobinuria, discusses the direction of continuing research in this area, as well as the potential for the development of management guidelines. It serves as a valuable source of information for both basic scientists and physicians, especially immunologists targeting GPI-anchored proteins and complements, and hematologists specializing in bone marrow failure.
Paroxysmal Nocturnal Hemoglobinuria (PNH) has been recognized for over a century. This mysterious disease is now understood at the level of the gene and the protein. The pathophysiology is related to a class of cell surface proteins with distinctive biochemical and physical characteristics. Recently it has been acknowledged that PNH is not rare, and once sensitive assays--based on the chemistry of the proteins--can be applied to many patients.Written by international experts in the field, this book includes a number of distinctive characteristics, such as the clinical features of PNH, the mechanism of hemolysis, the biochemistry of glycosylphosphoinositol anchors, and the chemistry and biophysics of GPI-anchored proteins.This unique and timely volume will have a wide audience, including hematologists and oncologists with a clinical interest in this disease, as well as basic biochemists, immunologists, and cell biologists studying this class of proteins. - Outlines the chemical features of PNH - Explains the mechanism of hemolysis - Includes work on the biochemistry of glycophosphoinositol anchors - Contains descriptions of the chemistry and biophysics of GPI-anchored proteins
This book provides a comprehensive account on individually rare, but collectively frequent diseases of the liver. In the first part, conditions such as hepatic vascular malformations, ischemic cholangiopathy, hepatic artery occlusion, sinusoidal conditions and Budd-Chiari syndrome are discussed among others. The second part examines the causes of vascular liver disease namely, coagulation disorders, neoplasm disorders, non-malignant blood disorders, systemic diseases and toxins, among others. The unique expertise of the authors, who are all members of the Vascular Liver Disease Disorders Group, an independent network of researchers with a common interest in Vascular Liver Diseases, are merged for an optimal pragmatic and individualized approach. This book is of interest to a broad range of experts, such as hepatologists, internists, radiologists and angiologists.
Congenital and Acquired Bone Marrow Failure is a comprehensive guide to congenital and acquired bone marrow failure in adult and pediatric patients. Chapters are divided into two sections, acquired aplastic anemia and inherited bone marrow failure syndromes. Content ranges from the basic, to the translational, and from the epidemiology of acquired aplastic anemia and telomere biology, to the management, treatment, and supportive care of pediatric, adult, and geriatric patients. Contributors are world leading experts in the field of bone marrow failure. The book is required reading for residents, fellows, clinicians, and researchers across hematology, oncology, pathology, bone marrow transplantation, pediatrics, and internal medicine.
Handbook of Benign Hematology is a practical guide to the diagnosis and management of benign hematologic conditions. The book begins with a chapter on normal hematopoiesis and follows with chapters devoted to groups of blood disorders and syndromes including neutrophil disorders, nonmalignant myeloid disorders, bone marrow failure syndromes, myeloproliferative disorders, anemias, iron metabolism disorders, platelet disorders, hemostasis and coagulation defects, and thrombosis. Each disorder subtype covered features a clinical case, an introduction to the condition, details on diagnosis including applicable criteria and lab work needed, key diagnostic dilemmas, prognosis, treatment options, details on clinical trials and emerging clinical strategies, and bulleted key points to highlight clinical pearls and common pitfalls. The final chapters provide best practices for transfusion medicine and a guide to pharmacologic agents and their uses in clinical practice for adult and pediatric patients. The handbook is filled with tables and illustrations which highlight FDA-approved drug information, clinical trials data, hematopathologic characteristics of different disorders, important management criteria and more, making it the ideal handbook for those in practice or for review. The Editors and chapter authors are experienced academic practitioners in the fields of adult and pediatric hematology, pathology, blood banking, and pharmacology. Emphasizing best practices for patient management, this handbook is essential for oncologists, hematologists, trainees, and other practitioners who regularly or increasingly receive referrals to diagnose and treat adults or children with nonmalignant hematologic conditions. Key Features: Includes dozens of clinical cases covering all nonmalignant blood disorders Emphasizes patient management and best practices for disorders seen in adults and children Contains over 30 color images and numerous tables for quick reference Presents important details of all pharmacologic agents used to treat or manage hematologic disorders and their complications Purchase includes access to the ebook for use on most mobile devices or computers
This text addresses the need for a book specifically aimed at obstetric anesthesia and covers topics such as pulmonary, cardiac renal, hepatic, hematologic, neurologic, endocrine and other diseases. The real anesthetic challenge arises when patients present to Labor and Delivery with unusual or complicated medical problems and, in recent years, a few of the larger institutions have developed an Obstetric Anesthesiology Consultation Service to prepare for the management of these patients. While most pregnant women who present to Labor and Delivery require anesthetic intervention, they typically meet the anesthesiologist for the first time in labor. Since the majority of laboring women are healthy without significant comorbidities, this does not present much of a challenge to the anesthesiologist and the anesthetic management tends to be straight-forward with favorable outcomes. However, using this new model, the anesthesiologist has the opportunity to discuss the various treatment modalities and potentially suggest diagnostic testing to be performed prior to delivery, similar to the pre-operative testing that is done in other surgical environments.
This book offers a systematic introduction to Budd-Chiari syndrome, a rare but life-threatening vascular disorder of the liver, which refers to obstructions at any site from the hepatic veins to the suprahepatic inferior vena cava. The book mainly covers the history, epidemiology, etiology and risk factors, pathology, diagnosis, pharmacological and interventional aspects, surgical treatment, etiological treatment, and prognostic assessment of Budd-Chiari syndrome. Notably, it also presents cutting-edge research findings in this field. The early diagnosis of Budd-Chiari syndrome is vital, as it allows clinicians to pursue more targeted and effective treatment strategies, thus improving patient outcomes. However, much information, including the latest advances, has not yet been fully disseminated in current clinical practice. This book addresses that gap, helping physicians to improve diagnosis and establish standard treatment strategies, helping researchers conduct clinical and experimental studies, and even helping patients and their relatives to recognize this disease.
