This volume provides a comprehensive overview of critical care of the pediatric immunocompromised hematology-oncology patient. The text focuses on unique aspects of the pediatric immunocompromised patient that predisposes the child to significant illness, and presents critical care management strategies specific to the patient population. In addition to chapters on oncology, primary immune deficiency, immunocompromised hematology, and hematopoietic cell transplant patients, the book covers the changing landscape of ICU care, pharmacologic considerations, and psychological and social aspects of the critical care of hematology-oncology patients. Written by experts from a range of disciplines, Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient: An Evidence-Based Guide is a valuable resource for clinicians and practitioners who treat this patient population.

Ayalew Tefferi, MD, and a team of authoritative practicing physicians concisely detail their most effective approaches to the diagnosis and treatment of a wide variety of hematological disorders, including anemia and other cytopenias, primary and secondary myeloproliferative disorders, lymphoid and plasma cell disorders, and thrombotic and bleeding disorders. For each illness these expert clinicians provide a brief review of basic concepts and a discussion of its symptomology and classification, its diagnostic tests, and strategies for its management, along with suggested readings for more detailed information. Numerous decision trees clarify diagnosis, and summary boxes highlight key points and dosage parameters. Concise, practical, and up-to-date, Primary Hematology offers busy primary care physicians, internists, hematologists, and oncologists a much-needed action-oriented summary of proven approaches to the optimal diagnosis and treatment of hematologic disorders today.

Myelodysplastic syndromes (MDS) are the most common hematological malignancies involving mostly the elderly population. The major morbidity relates to patients' symptomatic cytopenias.MDS was previously named as "preleukemia " or " smoldering leukemia" as the lack of terminal cells in MDS and because about 25% of all cases progresses into acute myeloid leukemia. According to various reports the annual incidence of MDS ranges widely from 2-12 per 100.000, increasing to 30-50 cases per 100.000 among persons aged 70 or older. It is believed that the true incidence of MDS have been underestimated however it seems to be comparable to that for multiple myeloma and chronic lymphocytic leukemia. In the past decade much progress had been made; we know more on the disease pathology, there is more emphasis on the care and more targeted therapy had been invested. Athors provide updated knowledge in this book on all clinically important aspects of the disease. Hot topics of our days are discussed in chapters by outstanding and well known scientists from all over the world. We would offer this product both for medical students and postgraduates as well as for all who are interested in this very exciting and fast progressing field of hematology. With this work authors should call attention on the disease for decision makers in healt care systems as well.

Haemoglobin is one of the most important molecules in the animal kingdom. Its function is to carry oxygen to tissues. In lower invertebrates the blood pigment is present in the haemolymph and is not bound in cells. Later in the course of phylo genesis haemoglobin remains associated with cells which produce it and in this form it reaches the peripheral circulation. In higher organisms the haemoglobin production is thus determined by two main factors: haemoglobin synthesis in erythroid cells and the formation of these erythroid cells which depends on cell proliferation in haematopoietic organs. Human haemoglobin is made up of two chains which combine from four different polypeptide chains formed in varying ratios in different periods of the life cycle. During the life span of humans the following haemoglobins are formed: embryonic haemoglobins Gower 1 and 2, foetal haemoglobin F and two adult haemoglobins A and A . E-and IX-chains are part of the embryonic haemoglobins Gower 1 (E4) and 2 Gower 2 (1X2E2). These haemoglobins predominate in embryos during the second month of pregnancy and at the end of the first trimester they are completely re placed by foetal haemoglobin F ( Y2). Adult haemoglobin A consists of two IX and two -chains and is the main component of red cells in adults. A relatively small component of red cells accounting for less than 2 % of the total haemo globin, is haemoglobin A2 (1X0)."

WEGENER'S GRANULOMATOSIS & ANCA-ASSOCIATED DISEASES: THE STORY CONTINUES The disease now designated as Wegener's granulomatosis (WG) was first described in 1931 by Heinz Klinger, who considered it to be a special form of polyarteritis nodosa. Klinger's friend, Friedrich Wegener, expanded on the first observations and interpreted the pathological and clinical fmdings to represent a distinct disease entity (Wegener, 1939). He described this entity as a "peculiar rhinogenous granulomatosis with a unique participation of the arterial system and the kidneys". Later, Godman and Churg (1954) established the classical diagnostic criteria (the "WG triad"): granuloma, vasculitis, and glomerulonephritis. In 1958 Walton pointed out the poor prognosis of WG based on a small number of published cases (mean survival time: 5 months). In 1966 Carrington and Liebow reported "limited forms" of WG with a defmitely more favorable prognosis. Since then positive results have been reported with cyclophosphamide therapy. In addition, a retrospective study of combined low-dose cyclophosphamide and prednisolone in 85 WG patients over a period of 21 years found a similarly encouraging outcome. The*latter experience led to the current "standard" treatment protocol (FAUCI et al. , 1973 and 1983). More recently, strong evidence has emerged that some of the morbidity and mortality ofWG - and other types of systemic vasculitis - may be a consequence of this treatment (Hoffman et al. , 1992).

This new edition of an essential text for all those working within transfusion and blood banking is now even more biologically and clinically relevant, incorporating the latest information on the genes for various blood groups and including greater content on the functional significance of blood groups. The book covers techniques used in blood grouping, troubleshooting and quality assurance and integrates serology with molecular biology, marrying the basic understanding at the genetic level with a cellular understanding of the red blood cell membrane. Now in full colour throughout.

Transferring hematopoietic stem cells and immune cells has continued to be a promising therapeutic alternative and a fascinating area of cell biology as well as a field of persistent procedural problems. This explains why substantial parts of basic research on cell growth and differentiation, immune tolerance and antitumor effects, gene transfer, minimal residual disease and supportive care have settled around clinical transplantation in hematology and oncology. This second volume updates the current role of allogeneic and autologous transplantation in leukemias, lymphomas and solid cancers, including controversial strategies and novel experimental approaches. Outstanding representatives of leading groups guarantee first-hand information and indicate how we can work and cooperate more effectively to the benefit of our patients.

Since the first concepts of gene therapy were formulated, the hemopoietic system has been considered the most natural first target tissue for genetic manipulation. The reasons for this include the fact that a very large number of inherited disorders (including some of the most common disorders, such as the hemoglobinopathies) are disorders of the hemopoietic system, and the large amount of experience in hematopoietic transplantation biology. The consequence of this resulted in the first clinical trial of gene therapy in 1989, where two children suffering from severe combined immune deficiency (ADA-SCID) were transplanted with T-cells express ing adenosine deaminase (the defective enzyme in patients with this disorder). The partial success of this treatment was perhaps responsible for undue optimism among those proposing other gene therapy treatments within the hematopoietic system, and it has since become clear that there are a number of technical and biological difficulties to overcome before hematopoietic gene therapy becomes a mainstream therapeutic strategy. The chapters in this book evaluate the need for gene therapy in the hematopoietic system, discuss how efficient gene transfer and expression can be achieved in the target cells, highlight areas of difficulty to be addressed, and examine a number of potential applications of the gene therapy approach. The book begins with a chapter by Testa and colleagues, discussing the various sources of hematopoietic cells for both transplantation and gene therapy."

This series presents reviews covering all aspects of haemodynamics and haemorheology. Topics covered include the complexities of microcirculation, the rheology of blood and blood vessels, and the mechanics of blood flow in arteries and veins. The contributions aim to reflect the advances being made in experimental techniques and instrumentation for laboratory and clinical measurements and in numerical and mathematical modelling. Emphasis is placed on the scientific and engineering principles involved, but particular attention is also given to the clinical significance of this area of research. Topics covered by this volume include viscoelastic properties of blood and blood analogues; blood flow through narrow tubes; and numerical modelling of blood flow.

In September 1998 experts from 19 countries came together for an interdisciplinary discussion of the function of animal peroxidases, a family of enzymes embracing myeloperoxidase, eosinophil peroxidase, thyroid peroxidase and lactoperoxidase. Their papers have been updated for publication, yielding a wide-ranging overview of the state of the art. The chapters cover a wide range of topics, including three-dimensional structure of representative family members, their biosynthesis and intracellular transport, mechanism of action as well as applications to clinical medicine. They are of clinical relevance in, for example, arteriosclerosis, multiple sclerosis, infections, tumorigenesis, rheumatic diseases and hypothyroidism. This book forms an excellent introduction for anyone interested in the peroxidase family of enzymes.

A cutting-edge review of the latest findings on the complexities of platelet function and the various means of inhibiting platelet clot formation. The authors delineate an up-to-date picture of platelet biology and describe methods for assessing platelet function, including the commonly used platelet aggregation, thromboxane production, procoagulant function, platelet function under flow, and the expression of platelet activation markers. The focus is both on the technology and the outcome of research on platelets, including the fast developing fields of proteomics and genomics and their application to platelet research. The clinical applications of the various methods for the assessment of platelet function in vivo, as well as antiplatelet therapy, are fully discussed.

Are you constantly exhausted? Does sleep not refresh you? Is your balance not what it was? Do you have tingling or even burning in your fingers and toes? Then your problem may be vitamin B12 deficiency. Your doctor may test you for this but your blood levels look OK so what should you do then? Or you may receive treatment but not feel any better? This book is a guide to the complexities of this deceptively simple problem - how it can be diagnosed, how it can be treated, and how those who have it can cope with the lifelong repercussions. Incorporating the latest research, and the input of the thousands of members of the Pernicious Anaemia Society, this book is both practical and engaging, illustrated with many personal stories that will resonate with sufferers and their friends and families.

The Sentinel Lymph Node Concept is evaluated from the points of view of pathology, radiodiagnosis and nuclear medicine diagnostics, surgical treatment and clinical oncology. The concept and its practical applications are analyzed for breast cancer, malignant melanoma, tumors of the face, oropharynx, lung, gastrointestinal and urogenital tracts. The first part of the book describes the function and use of the nuclear medicine equipment, the tracers used, colloid solutions and modern developments in histological and immunohistochemical lymph node investigations, as well as possible pitfalls. The consequences of false-negative results are clearly delineated. In the second part, specific tumor-related problems are described.

In the last decade, there has been a remarkable explosion of knowledge in hematologic cancer from basic molecular biology and pathology to clinical therapy. This has led to many new advance and insights in the understanding of pathobiology of malignant hematology. New knowledge of disease molecular pathology, cytogenetic, epigenetic and genomic alterations have provided new strategies to attack and eradicate tumor cells at molecular level and significantly impacted our current therapeutics for hematological malignancies. The recent and ongoing rapid expansion of knowledge in this area has become extensive, dynamic and diffuse over the literature and research publications. This has led to the need to capture and compile the new and current information about hematologic cancer with special emphasis on translation from molecular pathobiology to targeted therapeutics. In this book experts from around the world share their thoughts and knowledge about the pathobiology of hematologic cancer, as well as their view on current treatment approaches and future development in these malignant hematologic diseases. This book is well suited for hematology residents, fellows and hematology-oncology physicians, hematopathologist as well as basic research scientist in the area of hematologic malignancies.

Sickle cell and thalassaemia are among the world's most common genetic conditions. They are especially common in Africa, Brazil, the Caribbean, the Middle East and Asia. They affect all ethnic groups but they particularly impact on minority ethnic groups in North America, Europe and Australasia. Much research has focused on clinical, laboratory and genetic studies of these conditions. Through a wide-ranging selection of readings based on social scientific research into sickle cell and thalassaemia, this book seeks to redress this imbalance. This is important as, through an examination of the different social, economic and cultural contexts of the lives of people living with sickle cell or thalassaemia, the contributors demonstrate that people are more than the sum of their genes and that their life experiences are rarely derived solely from the clinical severity of their condition but depend on the social context of their lives. Genetics and Global Public Health presents a new concluding chapter which highlights the critical nature of social science research for sickle cell and thalassaemia communities, providing key insights into the social contexts of human behaviour and analysing how societal arrangements could change to assist people living with either condition. It will be of great interest to postgraduate and research students as well as professionals working in the field of public health. This book was originally published as a special issue of the journal Ethnicity and Health.

Ideal as a quick, easy-to-use reference in the laboratory or clinical setting, Atlas of Diagnostic Hematology is an abundantly illustrated guide to the vast range of malignant and non-malignant disorders of the blood. More than 1,200 vibrant, full-color images enable you to identify and compare the unique clinical and histologic features of various blood disorders and confidently reach a diagnosis. Coverage includes photos of actual cases that span the entire range of this complex field, including rare conditions and difficult diagnoses. Features more than 1,200 images including full-color pathologic and clinical images covering a wide range of hematologic malignant and non-malignant conditions. Covers a range of disease stages, from the slightest indication where a diagnosis can be complicated or missed entirely, to what the average blood or biopsy sample signifying disease may look like, to an advanced stage where disease indications are abundantly clear. Helps you distinguish between similar and overlapping features and symptoms to arrive at an accurate diagnosis. Provides up-to-date information on infectious processes in blood and bone marrow, classification system of myeloid neoplasms, and indolent and aggressive mature T and NK-cell lymphomas. Includes diagnostic algorithms with differential diagnoses for conditions with similar histologic features and clinical symptoms. Contains the latest WHO classifications for pathologic, genetic, and clinical information. Enhanced eBook version included with purchase. Your enhanced eBook allows you to access all of the text, figures, and references from the book on a variety of devices.

The hypoxia volumes will focus on cutting edge research at the interface of hypoxia and biomedicine. Hypoxia is a constant threat to the human body and its vital organs throughout life. There are many situations in which the threat is heightened in health and disease, but mechanisms have evolved to lessen its detrimental effects. The International Hypoxia Symposia was founded to enable scientists, clinicians, physiologists, immunologists, mountaineers and other interested individuals to share their experiences of the situations associated with oxygen lack and the adaptations that allow us to survive. The mission of the International Hypoxia Symposia is to present cutting edge, sophisticated research at the very highest levels into the many effects of hypoxia on humans and animals in health and disease.

Expertly edited and endorsed by the International Society for Laboratory Hematology, this is the newest international textbook on all aspects of laboratory hematology. Covering both traditional and cutting-edge hematology laboratory technology this book emphasizes international recommendations for testing practices. Illustrative case studies on how technology can be used in patient diagnosis are included. "Laboratory Hematology Practice" is an invaluable resource for all those working in the field.

In this issue of Hematology/Oncology Clinics, guest editors Drs. Matthew B. Yurgelun and Douglas A. Rubinson bring their considerable expertise to the topic of Pancreatic Cancer. Top experts in the field cover key topics such as pancreatic adenocarcinoma: trends in epidemiology, risk factors, and outcomes; decision making regarding perioperative therapy in individuals with localized pancreatic adenocarcinoma; the evolving role of radiotherapy in the management of individuals with pancreatic adenocarcinoma; and more. Contains 14 relevant, practice-oriented topics including diabetes, cachexia, sarcopenia, and metabolic factors in individuals with pancreatic adenocarcinoma; PARP inhibitors and other novel therapeutics in pancreatic adenocarcinoma; screening and surveillance for pancreatic adenocarcinoma in high-risk individuals; germline testing for individuals with pancreatic adenocarcinoma and novel genetic risk factors; and more. Provides in-depth clinical reviews on pancreatic cancer, offering actionable insights for clinical practice. Presents the latest information on this timely, focused topic under the leadership of experienced editors in the field. Authors synthesize and distill the latest research and practice guidelines to create clinically significant, topic-based reviews.

Basophils and mast cells are similar but unique secretory cells with a well-documented role in immediate-hypersensitivity reactions. The presence of these cells in various cell mediated hypersensitivity reactions, in tissues of multiple diseases, and as a component of the host reaction to injury and repair in numerous circumstances is well known. Release of stored and newly generated mediators of inflammation from basophils and mast cells contributes to the cascade of pathogenetic events in circumstances under which these release reactions occur. Despite insights acquired through studies of these pathologic events, the role of basophils and mast cells and their secretory products in health is not known. In this book, I review much of the structural information regarding basophils and mast cells of multiple species. Ultrastructural studies of rat mast cells historically precede and quantitatively exceed similar studies of basophils and mast cells of other species. Therefore, I first review these background studies as an entity. Then I discuss the contents of two prominent organelles-granules and lipid bodies-in basophils and mast cells of several species. The ultrastructural morphology of basophils and mast cells in three species is presented in detail to establish appropriate guidelines for their recognition and to provide general rules for analysis which are appropriate for the identification of these cells in other species as well."

In this issue of Hematology/Oncology Clinics, guest editors Drs. Sung-Yun Pai and Nirali N. Shah bring their considerable expertise to the topic of Gene-Based Therapies for Pediatric Blood Diseases. Top experts in the field cover key topics such as CAR T-cell therapy: current status; engineered T cells; NK-cell therapy; hemoglobinopathies: beta-thalassemia, sickle cell disease; hemophilia A/B; primary immunodeficiencies; and more. Contains 14 relevant, practice-oriented topics including the evolution of gene therapy; viral vectors in hematopoietic stem cell gene therapy; gene editing in hematopoietic stem cells; nonintegrating vectors and engineered capsids; regulatory aspects of gene therapy; and more. Provides in-depth clinical reviews on gene-based therapies for pediatric blood diseases, offering actionable insights for clinical practice. Presents the latest information on this timely, focused topic under the leadership of experienced editors in the field. Authors synthesize and distill the latest research and practice guidelines to create clinically significant, topic-based reviews.

Pulmonary embolism is a common but vexing illness. This book provides a contemporary overview of the most important issues from a Western and a Japanese perspective, providing the reader with state-of-the-art knowledge of the epidemiology and molecular biology of the disease. In the Diagnosis section, the relationship between venous thrombosis and pulmonary embolism is explored, and exciting new findings are presented in the fields of echocardiography and magnetic resonance imaging. The Management section focuses on surgical intervention with acute embolectomy as well as updating thromboendarterectomy for chronic pulmonary embolism. The importance of primary and secondary prevention is emphasized in chapters addressing low molecular weight heparin and the inferior vena cava filter. This cutting-edge book is a valuable resource for practicing physicians, researchers, and students in internal medicine, hematology, pulmonary critical care, and vascular medicine.