Sickle cell anaemia is an inherited blood disorder, characterised primarily by chronic anaemia and periodic episodes of pain and occurring in approximately 1 in every 400 African-American infants born in the United States each year. Individuals of Mediterranean, Arabian, Caribbean, South and Central American, and East Indian ancestry can also be affected. The underlying problem involves haemoglobin, a component of the red cells in the blood. The haemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues and bring back carbon dioxide to the lungs. In sickle cell anaemia, the haemoglobin is defective. After the haemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures. These structures cause the red blood cells to become stiff and to assume a sickle shape. Unlike normal red cells, which are usually smooth and donut-shaped, the sickled red cells cannot squeeze through small blood vessels. Instead, they stack up and cause blockages that deprive the organs and tissue of oxygen-carrying blood. This process produces the periodic episodes of pain and ultimately can damage the tissues and vital organs and lead to other serious medical problems. Unlike normal red blood cells, which last about 120 days in the bloodstream, sickled red cells die after only about 10 to 20 days. Because they cannot be replaced fast enough, the blood is chronically short of red blood cells, a condition called anaemia. Sickle cell anaemia is caused by an error in the gene that tells the body how to make haemoglobin. The defective gene tells the body to make the abnormal haemoglobin that results in deformed red blood cells. This book gathers the latest research in this important field.

This text provides a thorough resource on arterial blood gases, covering the full scope of applications. This book is the first of its kind to focus on the needs of educators, students, and practitioners alike. The new edition has been completely updated, providing the latest information from the field, including facts on technical issues, basic physiology, clinical oxygenation, clinical acid base, non-invasive techniques, just to name a few. Instructor resources are available; please contact your Elsevier sales representative for details. This book's amazing content coverage offers a wealth of useful material, including illustrations, tables, examples, and case studies. This new edition is up-to-date with the latest in technology and information, ensuring the most current information is available. New figures and tables enhance the understanding of chapter material. The addition of an NBRC (National Board of Respiratory Care) Challenge at end of each chapter helps readers learn, understand, and put the information together to master the subject. The incorporation of two new On Call Cases per chapter provides further opportunity to practice clinical application of content learned, as well as helping readers utilize their critical thinking skills. Reorganized and improved table of contents presents the material in a more logical, efficient manner.

Blood is vital to most animals. In mammals it transports oxygen and food, carries away waste, and contains the white cells that attack invading microbes. Playing a central role in life, it has had profound cultural and historical significance and plays an important role in religious ritual. Blood was one of the four humours in early Western medicine and is still probably the major diagnostic tool in the doctor's armoury. In this Very Short Introduction, Chris Cooper analyses the components of blood, explains blood groups, and looks at transfusions, blood tests, and blood-borne diseases. He considers what the future may hold, including the possibility of making artificial blood, and producing blood from stem cells in the laboratory. ABOUT THE SERIES: The Very Short Introductions series from Oxford University Press contains hundreds of titles in almost every subject area. These pocket-sized books are the perfect way to get ahead in a new subject quickly. Our expert authors combine facts, analysis, perspective, new ideas, and enthusiasm to make interesting and challenging topics highly readable.

Now expanded with new coverage of genetics, more therapy and management strategies, and more references throughout, this guide remains one of the most practical resources for diagnosis and treatment of hematologic conditions commonly seen in general practice. The clear, succinct language is meant for the non-hematologist who wants the salient points of clinical signs, etiology and pathophysiology, laboratory tests, differential diagnosis, and treatment in one convenient manual.

Understanding the connections between culture, race, politics, and disease This groundbreaking book chronicles the history of sickle cell anemia in the United States, tracing its transformation from an ""invisible"" malady to a powerful, yet contested, cultural symbol of African American pain and suffering. Set in Memphis, where one of the nation's first sickle cell clinics was founded in the 1950s, Dying in the City of the Blues reveals how the recognition, treatment, social understanding, and symbolism of the disease evolved in the twentieth century, shaped by the politics of race, region, health care, and biomedicine. Using medical journals, patients' accounts, black newspapers, blues lyrics, and many other sources, Keith Wailoo follows the disease and its sufferers from the early days of obscurity before sickle cell's ""discovery"" by Western medicine; through its rise to clinical, scientific, and social prominence in the 1950s; to its politicization in the 1970s and 1980s. Looking forward, he considers the consequences of managed care on the politics of disease in the twenty-first century. A rich and multilayered narrative, Dying in the City of the Blues offers valuable new insight into the African American experience, the impact of race relations and ideologies on health care, and the politics of science, medicine, and disease.

Eisenstoffwechselstorungen, insbesondere der Eisenmangel, zahlen zu den am haufigsten ubersehenen oder fehlgedeuteten Erkrankungen. Anamien sind ein weltweites Problem. Davon betroffen sind vor allem altere Menschen. Nach den WHO-Kriterien (World Health Organisation. Nutritional anemias. Technical Reports Series 1992; 503) spricht eine Hb-Konzentration

This title includes proceedings of the first European Symposium on platelet immunology held in Paris, Palais du Luxembourg (France), 1 to 2 March 1990.

Today every ICU provides rapid and automated blood gas testing twenty-four hours a day. The emphasis in this handy manual on blood gases is on interpreting readings and wisely using the information derived. The self-testing questions and glossary make it particularly useful. The Second Edition includes patient scenarios, more figures, a revised bibliography, and pertinent Internet addresses. Compatibility: BlackBerry(R) OS 4.1 or Higher / iPhone/iPod Touch 2.0 or Higher /Palm OS 3.5 or higher / Palm Pre Classic / Symbian S60, 3rd edition (Nokia) / Windows Mobile(TM) Pocket PC (all versions) / Windows Mobile Smartphone / Windows 98SE/2000/ME/XP/Vista/Tablet PC

Lanzkowsky's Manual of Pediatric Hematology and Oncology, Sixth Edition, is a comprehensive book on patient management, replete with algorithms and flow diagrams on diagnosis and management. Reflecting the considerable advances in the treatment and management of hematologic and oncologic diseases in children, the sixth edition of this successful clinical manual has been entirely updated to incorporate all current treatment protocols, new drugs, and management approaches. Its concise and easy-to-read format will enable readers to make accurate diagnoses and permit them to treat patients without having to reference larger medical textbooks. Based on the new standards of genetic classification and prognostic information that have arisen in the past five years, the sixth edition includes two new chapters (Diagnostic, Molecular, and Genomic Methodologies for the Hematologist, Transfusion Medicine) and several new expanded chapters that were previously sections in consolidated chapters (Myelodysplasia, Myeloid Leukemias, Lymphoid Leukemias, Hemolytic Anemia, and Disorders of Coagulation).

Biomedical scientists are the foundation of modern healthcare, from cancer screening to diagnosing HIV, from blood transfusion for surgery to food poisoning and infection control. Without biomedical scientists, the diagnosis of disease, the evaluation of the effectiveness of treatment, and research into the causes and cures of disease would not be possible. The Fundamentals of Biomedical Science series has been written to reflect the challenges of practicing biomedical science today. It draws together essential basic science with insights into laboratory practice to show how an understanding of the biology of disease is coupled to the analytical approaches that lead to diagnosis. Assuming only a minimum of prior knowledge, the series reviews the full range of disciplines to which a Biomedical Scientist may be exposed-from microbiology to cytopathology to transfusion science. The science of transfusion and transplantation demands a multifaceted understanding of immunology, haematology, and genetics from the biomedical scientist. Transfusion and Transplantation Science synthesizes the essential concepts of these subjects and presents them within the practical framework of the hospital banking and transplantation centre, providing you with the knowledge and skills to specialize in this discipline.

A Guide to Paediatric Red Blood Cell Disorders is a comprehensive text on common red blood cell disorders encountered in children. It is a useful guide to postgraduate doctors training in paediatrics and haematology, medical undergraduates, primary care physicians and practising clinicians. The book is divided into five sections. The first section provides a detailed understanding of the basic concepts and approach to red blood cell disorders in children. This section includes information on the structure and function of red blood cells and haemoglobin, epidemiology and aetiology of anaemia and clinical and laboratory evaluation of childhood anaemia. The next three sections will provide information on paediatric conditions that result in microcytic, normocytic and macrocytic anaemia, respectively. The final section will be on conditions leading to polycythaemia in neonates and children. Throughout the book, the emphasis is given to common conditions that are frequently encountered in clinical practice. However, rare but clinically important conditions have also been included. Each chapter is divided into subheadings to describe the epidemiology, aetiology, genetic basis, molecular pathology, pathophysiology, classification, clinical features, investigations, diagnosis, treatment, follow-up and prognosis of each disorder. At the end of each chapter, a section on recent advances provides information on promising novel developments and experimental approaches for treating these diseases. This book will help medical undergraduates to grasp concepts and understand the entire spectrum of red blood cell disorders in children. For practising clinicians, this will be a useful guide on how to approach a child with anaemia, which is one of the most common presentations to general practice, field clinics and hospitals. For postgraduate doctors training in paediatrics and haematology, the book will provide comprehensive information on how to manage common as well as complex red blood cell disorders in children. This book is concise, reader-friendly and written in simple English, which can be understood by non-native speakers. It will aid readers across the globe to grasp the concepts of paediatric red blood cell disorders easily and be knowledgeable and up to date in managing these patients.

Text in French.

Intricate processes involved in perpetuating the multitude of physiological phenomena in the human body often encounter aberrations and omissions in the genetic code of life. While such errors often lead to lethal diseases, at other times they provide distinctive survival advantages and thus unscramble cues to unconventional therapeutic strategies for life-threatening conditions. Hereditary Persistence of Fetal Hemoglobin (HPFH) is one such condition wherein the typically inactivated fetal form of hemoglobin (HbF) remains overexpressed even in adult stages of the bearer's life. Strikingly, this condition is known to ameliorate pathological manifestations in patients with aberrant adult hemoglobin synthesis (e.g. I(2)-hemoglobinopathies like I(2)-thalassemia, sickle cell disease etc.). Early researchers in the field expected such patients to suffer from clinical challenges owing to HbF's high affinity to oxygen and consequent difficulty in its release to cells and tissues. Surprisingly, these patients are known to lead a physiologically normal life. Modern-day hematologists and clinical researchers have looked-up to the concept of "HbF reactivation" as a potential curative strategy for patients suffering from I(2)-hemoglobinopathies like I(2)-thalassemia and sickle cell disease. As a result, several drugs like hydroxyurea, 5-azacytidine, cytosine arabinoside, natural products etc. have been tried in clinics to elevate HbF levels in such patients with limited success and poor understanding on the mechanisms of their action. Associated side-effects and complications of using cytotoxic agents like these restrict their use in most instances. Fortunately, with the advent of newer molecular tools and techniques, researchers are focusing their attention to reengineer the molecular machinery and thus reactivate the gamma-globin gene. This book brings together a selection of chapters dedicated to fetal hemoglobin a its physiological role, regulation, methodologies to manipulate and future strategies. Researchers and scientists interested in the topic will have a comprehensive understanding of the current concepts on fetal hemoglobin modulation and therefore will serve as a launching pad for their research ideas.

This issue of Hematology/Oncology Clinics, Guest Edited by Drs. Rachael Grace and Russell E. Ware, will focus on Pediatric Hematology. This issue is one of six selected for the year by the series Consulting Editors, George P. Canellos and H. Franklin Bunn. Topics include, but are not limited to, Rare Congenital Hemolytic Anemias, Sickle Cell, Thalassemia, Neutropenia and rare leukocyte disorders in children, Primary and Secondary Immune cytopenias, Disorders of Iron overload, Disorders of Iron metabolism, Approach to Hemophilia in a Changing Treatment Landscape, Von Willebrand disease, Inherited platelet disorders, Thrombosis, Diagnostic evaluation and medical approach to complications and treatment of vascular anomalies, New approaches and trials in Transfusion Medicine, and Updates in Neonatal Hematology.

This issue of Hematology/Oncology Clinics, guest edited by Patrick A. Ott, will focus on Immunotherapy in Cancer. Topics include, but are not limited to, Cancer Vaccines, Innate Immune stimulation, Costimulatory and Agonistic Antibodies, Immune modulation with radiation, Oncolytic virus therapy, Cytokine Therapy, Adoptive T cell transfer, Immune related toxicity, and Immune checkpoint combinations.

The authors summarise advances in human pluripotent stem cells-derived erythroid development and molecular regulatory mechanisms. This research may provide a new perspective on human embryo erythropoiesis and a possible treatment for some hematological diseases. Erythrocytes are well equipped to carry out their functions due to a dynamic cell membrane, their inherent shape and lack of organelles and cytoplasmic viscosity. As such, the following section focusses on the causes of these modifications and their clinical implications. As an example of complexity in research towards the development of erythrocyte membrane-based drug delivery systems starting from animal erythrocyte, morphological, biochemical and drug release profiles will be reviewed in the penultimate chapter. The final chapter investigates the electrochemical behavior of erythrocytes at platinum, carbonaceous, and optically transparent electrodes via polarization and coulometric measurements. The order of magnitude of the quantity of electrons transferred between erythrocytes and electrodes was determined, and potential ranges showing indifference of the electrode toward red blood cells were identified.

In this book, world-renowned experts in the field express well-reasoned opinions on a range of issues and controversies relating to haploidentical transplantation with the aim of providing practicing hematologists with clinically relevant and readily applicable information. Among the areas covered are graft manipulation and methods to control T-cell alloreactivity, the nature of the ideal graft and donor, haploidentical transplantation in pediatric and adult patients with malignant and nonmalignant diseases, immunologic reconstitution following transplantation, complications, and the prevention and treatment of relapse post transplantation. Attention is drawn to the implications of high-impact clinical trials whenever such trials are available. The readily intelligible text is complemented by numerous helpful tables, algorithms, and figures. The book will provide practical support for hematologists and transplant physicians as they attempt to provide optimal care in this exciting but increasingly complex medical specialty.

Prevailing physiological concepts (PPC) of blood circulation consider the cardiovascular system (CVS) an autonomous system that has its own goal and mechanisms for achieving it. Physiologists agreed that complex neural and humoral controllers of a mean arterial pressure (MAP) indirectly alter the blood flows for satisfying cellular needs. However, PPCs are incapable of explaining the causes of long-term shifts of an MAPs rest level. In particular, this affects current understanding and cure technologies of arterial hypertension (AH). Considering AH as a disease, physicians seek a cure that effectively decreases the elevated pressure. This gave rise to the palliative cure softening of AH symptoms without an understanding of AHs primary causes. But this strategy, working until the patient intakes antihypertensive drugs, often leads to AHs further development, and in extreme cases current antihypertensive drugs are helpless. These limitations of PPC are forced to seek a circulations extended physiological theory (EPT), explaining the mechanisms of both normal and altered MAPs. In the EPT presented in the book, CVS is considered a constituent part of a more complex functional super-system (FSS) that appeared in a multi-cellular animal organism during the co-evolution of specialized cells. The general goal of the FSS is to provide optimal physiochemical and energy states of the cell cytoplasm. To achieve this goal under a stochastic total and local variations of cells activity, FSS should control: i) The cardiac output; ii) the regional and local blood flows; and iii) the chemical composition of both arterial and venous blood. Under chronic energy shortage, FSS should also provide an adequate increasing of ATP-synthesis in mitochondria of stagnated cells. So, under the ineffectiveness of current mitochondria, FSS must enrich the arterial blood by chemicals providing the biogenesis of mitochondria. However, neither the energy providers nor the providers of blood chemistry are properly involved in PC of the blood circulation. The EPT for the first time integrates the hemodynamic and metabolic aspects of cell life at the organism scale. It is proved that the CVS activity is inversely associated with the activity mechanisms controlling the rates of both pulmonary ventilation and erythropoiesis. Under significant and chronic energy deficiency, the cells activate additional FSS mechanisms, materially supporting the biogenesis of mitochondria. The activity of FSS mechanisms forming the chemical composition of arterial and venous blood is in reciprocal relationships with the function of CVS. So, the EPT associates the function of CVS with energy and metabolic problems in cells. The EPT concerns both traditional and additional determinants of the MAP level. It is proved that stochastic combinations of these determinants force the MAP level to float. In particular, both the mitochondrial insufficiency and the chemical contamination of cytoplasm are capable of causing AH. The normal arterial pressure is always individual. Before correcting the altered arterial pressure, a complex medical examination for ascertaining the mitochondrial function, the status of the FSS mechanisms is recommended. The diagnosis of AH should be reoriented for detecting cellular abnormalities. The therapy of AH should be targeted at finding strategies for the optimizing the entire FSS function.

Cyclic AMP was a major molecule of interest, which played an important role as second messenger, contributing to signal transduction in the regulation of cellular function by peptide hormones. Afterwards, calmodulin and protein kinase C were discovered as modulator proteins of intracellular calcium signaling in hormonal action. After that, manifold proteins and their related molecules were demonstrated to participate in novel signaling pathways related to various cytokines in different types of cells. The author of this book discovered a novel protein known as regucalcin, which suppresses manifold signaling pathways related to transcription activity. After subsequent studies, regucalcin has been established to play a pivotal role in maintaining cell homeostasis and protecting it from disorders in various types of cells and tissues. This book will provide information regarding regucalcin that plays a pivotal role in cell homeostasis and disorder. This book is composed of eighteen chapters. These chapters include the following content: the discovery of regucalcin (Chapter One); chemical property and structure of regucalcin (Chapter Two); the regucalcin gene and its translational regulation (Chapter Three); the role of regucalcin in intracellular calcium homeostasis (Chapter Four); the role of regucalcin in cell nuclear function (Chapter Five); the role of regucalcin in protein synthesis and proteolysis (Chapter Six); the suppressive role of regucalcin in cell proliferation (Chapter Seven); how regucalcin protects apoptotic cell death (Chapter Eight); the protective role of regucalcin in oxidative stress (Chapter Nine); the involvement of regucalcin in liver metabolic disorder (Chapter Ten); the role of regucalcin in kidney cell homeostasis: involvement in renal failure (Chapter Eleven); the role of regucalcin in heart calcium signaling: insight into cardiac disorder (Chapter Twelve); the role of regucalcin in brain calcium homeostasis: disorder with aging (Chapter Thirteen); the role of regucalcin in bone homeostasis and osteoporosis (Chapter Fourteen); the role of regucalcin in lipid metabolism and diabetes (Chapter Fifteen); the role of regucalcin as a suppressor protein in carcinogenesis (Chapter Sixteen); the clinical aspects of regucalcin as a biomarker for disease (Chapter Seventeen); and conclusive remarks (Chapter Eighteen). This book will provide information regarding regucalcin and its pivotal role in cell homeostasis and disorder.

The subject matter of volume 1 of the 2-volumes-handbook focusses on leiomyosarcoma, low-grade and high-grade endometrial sarcoma and undifferentiated uterine sarcoma of the whole female genitalia. A separate extensive chapter is devoted to the variants of leiomyoma (angio-, lipo-, cotyledonoid, cellular, mitotically active, epithelioid and myxoid leiomyoma, leiomyoma with bizarre nuclei), atypical smooth muscle tumors (smooth muscle tumors with uncertain malignant potential), and disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, and intravenous leiomyomatosis. Furthermore, endometrial stromal tumors - endometrial stromal nodules, endometrial stromal tumor with sex cord-like elements (ESTSCLE), uterine tumor resembling ovarian sex-cord tumor (UTROSCT) - and similar tumors are described in detail. The book provides a description at length of the epidemiology, etiology, pathological anatomy, prognosis, diagnosis, differential diagnosis, imaging and comprehensive therapy of each primary, relapsed, and metastasized tumor including surgery, chemo-, hormone- and radio- and targeted therapy. Another chapter is devoted to the prevention of subjecting sarcomas to inadequate surgical therapeutic measures under the assumed diagnosis of leiomyoma, and includes a diagnostic-therapeutic flowchart with a diagnostic score. The book aims to identify and provide diagnostic and therapeutic guidance. The listed tumor entities also constitute a particular diagnostic challenge for pathologists that contains numerous pitfalls and difficulties. This book, therefore, addresses gynecologists and pathologists in both clinical and private practice, but also surgeons and hemato-oncologists.

Accurate analysis of blood gases is vital to give information on a patient's respiratory and circulation state as well as the adequacy of resuscitation. This book guides the reader, with the help of clarifying cartoons, through the basic principles and a new and easy to grasp system of interpretation.

Sickle cell hemoglobin (HbS) is the result of a single nucleotide change (GAG GTG) in the beta-globin gene, where valine replaces glutamic acid at the sixth amino acid position in the beta-globin chain. Sickle cell disease is a growing global health problem. The World Health Organization has estimated that 7% of the world population has the mutation and 300,000400,000 affected children are born every year. The disease progresses towards a severe chronic hemolytic anemia, and it shows a heterogeneous clinical course, related with different genetic factors. Despite the fact that all subjects with sickle cell disease (SCD) have the same single base pair mutation in the DNA, we further confirmed here that the severity of the clinical and hematological manifestations is extremely variable. Increasing evidence has indicated a role of oxidative stress in the vascular pathophysiology of SCD. The vascular endothelium is central to disease pathogenesis because it displays adhesion molecules for blood cells, balances procoagulant and anticoagulant properties of the vessel wall and regulates vascular homeostasis by synthesizing vasoconstricting and vasodilating substances.In addition, recent studies support the existence of a hyperoxidative status in SCD patients that may account, at least in part, for the clinical manifestations of these patients. Moreover, SCD patients with mild clinical outcomes were associated with low oxidative status, whereas high oxidative stress was related to severe phenotypes. Thus, the use of oxidative stress biomarkers may be important in the evaluation of the clinical condition of SCD patients, whereas the use of therapies to improve their redox status and increase NO bioavailability would be beneficial to reduce the severity of sickle disease. The global burden of SCD is now significantly increased and, thus, it is currently a public health problem around the world. This disease has passed from being a problem of the developing countries to affect many people in developed countries. This book summarizes the current epidemiology status and the latest discoveries in the pathophysiology of SCD, and the potential therapies that may improve the clinical course of this disease.

Transfusion Medicine is a key part of modern health care. It bridges the healthy community with the bedside in hospitals. It is the responsibility of the national blood program to provide an adequate supply of blood for all patients requiring transfusion, and to ensure the quality of blood and blood products for clinical use and the in-hospital transfusion chain. All products must be safe, clinically effective and of appropriate, and consistent quality. Every blood transfusion service, whether serving in a resource restricted environment or in an advanced ambience, should develop an effective quality (QS) and quality management system (QMS) to ensure the implementation of these strategies from vein to vein. The quality system and its management should cover all aspects of its activities and ensure full traceability (hemovigilance), from the motivation, mobilisation and selection of blood donors to the transfusion of blood and blood products to patients. It should also reflect the structure, needs and capabilities of the procurement establishments, as well as the needs of the hospitals and patients that it serves. Management commitment and support are essential for the development, implementation and monitoring of a national quality system and quality management system in order to ensure change management and continuous quality improvement. All staff should understand the importance of quality and the consequences of failure in the quality system (error management and cost effectiveness).

Neutrophil granulocytes are the primary defense cells of blood against bacteria, fungi, parasites, or thrombi. Their main weapons and signals are reactive oxygen species (ROS) that release photons. The activation of the assembly of their NADPH-oxidase, the few specific triggers and many specific or unspecific primers are of great physiological and pathophysiological importance in inflammation and in hemostasis. The neutrophils generate different types of photons and they can "see" them. The 300400 nm photons are the main signals and the photons of lowest wave length which seem to especially alert them in emergency. The present book presents research on the regulation of the neutrophil's ROS generation by different photons, by singlet oxygen (the excited "pro-drug" of photons), by important proteins, or by modulators of the eicosanoid metabolism that should not favor the generation of systemically circulating micro-thrombi.

Hyperglycemia is the central metabolic abnormality in diabetes mellitus and it serves as the basis for making the diagnosis in both type 1 and type 2 diabetes. In this book, the authors present topical research in the study of the causes, symptoms and treatment options for hyperglycemia. Topics discussed include the ethiopathogenesis of stress hyperglycemia and its observation in acute coronary syndrome (ACS); developmental programming of hyperglycemia and metabolic outcomes; the role of hyperglycemia in diabetic nephropathy; and management of hyperglycemia in diabetic vascular disease.