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).

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."

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.

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.

Clinical Laboratory Hematology continues as a comprehensive, yet easy-to-read text of hematology and hemostasis written for students at all levels in clinical laboratory science programs, including clinical laboratory technicians, CLT (medical laboratory technicians, MLT), and clinical laboratory scientists, CLS (medical technologists, MT).

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.

The subject matter of volume 2 of the 2-volumes-handbook focusses especially on rare sarcomas of the whole female genitalia. These entities include angiosarcoma, the different lipo- and rhabdomyosarcoma as well as newer entities like the PEComa. Furthermore, mixed mullerian tumors like the benign adenofibroma and the malignant adenosarcoma and carcinosarcoma 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. An own chapter is devoted to the problems of fertility and pregnancy in connection with all sarcomas, variants of leiomyoma, atypical smooth muscle tumors, disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis and endometrial stromal tumors - endometrial stromal nodules, endometrial stromal tumor with sex cord-like elements (ESTSCLE), uterine tumor resembling ovarian sex-cord tumor (UTROSCT) -and all mixed tumors - of the female genitalia. 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.

This book discusses the aspects of haploidentical transplants and will shed light on the debates and questions on this burgeoning field and timely topic. Donor selection, graft failure, minimal CD34+ cell requirement, and conditioning regimens used for haploidentical transplants will be written by expert authors dealing with this type of transplants. Approximately one third of the books' chapters cover logic and basic aspects; the remaining two thirds of the book discuss clinical aspects, outcomes, and future perspectives, thus providing a comphrensive discussion of the topic. Haploidentical transplantation is extremely timely, rapidly-changing area and increasing its use will decrease the need for time-consuming, expensive, unrelated donor search. Moreover, Haploidentical Stem Cell Transplantation brings a set of clear answers to questions of feasibility, advantages over unrelated transplants, cost effectivity and outcome..

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.

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.

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.

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.

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.

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."

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.

This comprehensive text is suitable for researchers and graduate students of a 'hot' new topic in medical physics.

Written by the world's leading experts, this book aims to present recent developments in plasma medicine, both technological and scientific, reviewed in a fashion accessible to the highly interdisciplinary audience consisting of doctors, physicists, biologists, chemists and other scientists, university students and professors, engineers and medical practitioners.

The book focuses on major topics and covers the physics required to develop novel plasma discharges relevant for medical applications, the medicine to apply the technology not only in-vitro but also in-vivo testing and the biology to understand complicated bio-chemical processes involved in plasma interaction with living tissues.

The author examines hemostasis in animals from all seven major vertebrate classes. Her research provides unique insights into the phylogenetic development of the various phases and components of hemostasis. This monograph is a valuable reference for students, researchers, and teachers of biology, zoology, veterinary science, and human medicine.

This book is a comprehensive and up-to-date compendium on all aspects of blood and marrow transplantation in children. After an introductory chapter describing the history of pediatric blood and marrow transplantation, subsequent chapters discuss pediatric-specific aspects of transplantation, including stem cell sources suitable for transplantation, preparative regimens, graft-versus-host disease, complications related to transplantation, and late effects. The role of blood and marrow transplantation in various specific pediatric diseases is then examined, and the closing chapter considers future directions. The authors are all internationally recognized experts and offer a largely evidence-based consensus on etiology, biology, and treatment. This handbook has far-reaching applicability to the clinical diagnosis and management of pediatric diseases that are treatable with blood and marrow transplantation and will prove invaluable to specialists, generalists, and trainees alike.