Fully revised and updated for its second edition, Paediatric Haematology and Oncology provides an easily accessible source of information about all of the basic principles of childhood cancer and leukaemia, and detailed specialist knowledge on how to care for children with those conditions. Featuring new chapters on the biology of childhood leukaemia and central nervous system tumours, the book includes a greater focus on the rapidly expanding research in the biology and genetics of childhood malignancy, as well as new clinical treatments and more detail on various tumour types. Logically split into 12 sections on different aspects of haematology and cancer to allow quick and easy reference, the book provides general principles of diagnosis and treatment, short- and long-term care, and oncological emergencies, before moving on to chapters on specific diseases. Normal values and useful websites are also included for reference. Chapters have been expanded to feature more clinical images to aid in diagnosis and interpretation, making this second edition an invaluable companion for the trainee and consultant in paediatric haematology and oncology.

Natural killer (NK) cells are important effector cells of innate immune system implicated in many physiological processes including elimination of cancer cells and virus infected cells. NK cells comprise a majority of large granular lymphocytes circulating in peripheral blood with a minority derived from T cell lineage. Even though NK cells were first described more than 40 years ago, it was not until the 1980s and 1990s when immunophenotyping was incorporated into clinical diagnostic methods and resulted in discovery of distinct disorders of large granular lymphocytes and NK cells. Since then, significant progress was made in our better understanding of immunophenotypic and genotypic characteristics, biology, functions as well as disorders of these cells. Most recently, clinical studies using NK-cell based immunotherapy have shown promising results in treatment of some of malignant diseases. Disorders of large granular lymphocytes and NK cells are rare comprising only about 1% of all lymphoid malignancies in western countries. The rarity of these conditions was a main reason that the progress in our understanding of pathogenesis and development of novel therapeutic approaches has been delayed compared to development in more common B cell lymphoid malignancies. The low incidence of these diseases and scarcity of prospective clinical trials also limit the availability of evidence based research literature as well as comprehensive reviews about NK cell disorders. Thus, the editors decided to take on the challenging task and summarize our current knowledge about malignant and benign diseases of large granular lymphocytes in this book based on the best available evidence. The editors selected topics most relevant to clinical practice in order to provide a useful guide for practicing physicians. Chapters describing four disorders (T-cell large granular lymphocytic leukemia, chronic lymphoproliferative disorder of NK cells, extranodal NK cell lymphoma and aggressive NK cell leukemia) incorporated into most recent 2016 revision of classification of lymphoid malignancies are separated into experimental, diagnositc and clinical parts for easier understanding and reading. We are aware of challenges and inherited limitations of any larger project like this one due to a rapid progress especially in the field of genomics, which may not be incorporated in this book before it is published. The editors and contributing authors would like to thank the publisher NOVA for their support.

Blood has long been an object of intrigue for many of the world's philosophers and physicians, and references to it have existed since the earliest studies of human anatomy. Herodotus of Halicarnassus, whose writings 500 years before the birth of Christ drew on stories collected during his widespread travels, was amongst the first to identify the ritualistic and medical significance of blood. However, despite this long established history, haematology as a medical specialty is relatively new. A History of Haematology: From Herodotus to HIV traces the history of haematology from biblical times to the present, discussing the major defining discoveries in the specialty, ranging from war as a catalyst for the development of new techniques in blood transfusion, to the medical response to the HIV/AIDS epidemic. In this beautifully illustrated and passionately rendered history of the field of haematology, Professor Shaun McCann traces the remarkable developments within haematology and the work of the scientists and pioneers central to these advances. This engaging and authoritative history will appeal to a wide audience including haematologists, nurses and other health care workers in haematology, as well as medical students, and general physicians with an interest in haematology.

Myelodysplastic syndrome (MDS) is a family of clonal haematopoietic stem cells disorders characterized by dysplasia, ineffective hematopoiesis and susceptibility to transformation to Acute Myeloblastic Leukaemia (AML) that are shown to be strikingly refractory to current therapeutic modalities. The first chapter of this book provides a detailed review of the risk factors, treatment options and prognosis of MDS. Chapter two studies the inflammatory and autoimmune nature of MDS. Chapter three discusses the pathogenesis of 5q-syndrome. Chapter four examines the genetic mutations identified in MDS and their significance. Chapter five reviews different prognostic factors and stratifications of risk in Argentinean patients with MDS. Chapter six discusses epigenetics and epigenetic therapy. Chapter seven introduces mild oral chemotherapy treatments for elderly patients with a higher-risk myelodyspastic syndrome. Chapter eight discusses lenalidomide treatment in lower risk myelodysplastic syndromes. Chapter nine analyzes the old and new-integrating prognostic models and mutational advances with epigenetic and cellular therapies for MDS. The last chapter studies the entropy evaluation of bone marrow biopsies in MDS.

Sickle cell disease (SCD) is a genetic disorder caused by an abnormality of hemoglobin. The disease is characterized by a chronic hemolytic anemia. The search for affordable and accessible medicines mainly from plants and having various modes of actions for managing SCD is a priority in Africa where the disease is endemic. The first chapter in this book reviews children with Sickle Cell Disease (SCD). The authors also present their research that shows that clinically, children with SCD behave differently regarding their genetics. The second chapter gives an overview of the current progress in research in calcium handling in red blood cells of sickle cell disease patients, followed by an outlook into the potential use of blockers of the cation channels for therapy of SCD patients. The third chapter reviews and validates the pharmacological relevance of "Gardenia ternifolia" and sustains the use of this herbal medicine in the management of SCD in traditional medical systems. The fourth chapter reviews the search and the development of antisickling herbal drugs in Africa, where Sickle cell disease (SCD) is an endemic. The last chapter reviews SCD and its impact on sexual functioning as well as relationship dynamics. Conclusions support the importance of social support and its far-reaching impact into the coping mechanisms of patients with chronic illness as well as quality of life.

Coagulopathy (also called clotting disorder and bleeding disorder) is a condition in which the blood's ability to clot (coagulate) is impaired. Acquired hemophilia is a rare disorder characterized by spontaneous bleeding in a patient with no previous personal or family hemorrhagic history. The first chapter explores the challenges associated with this disease, its unfamiliarity often causing a delay in diagnosis and therapeutic approaches. The second chapter focuses on lower gastrointestinal bleeding (LGIB) in children, describing the epidemiology, etiology and clinical management. The third chapter reviews the pathogenesis, risk factors and treatment strategies of coagulopathy in patients with craniocerebral injury. The next article examines Vitamin B12 with its four cobalamin vitamers, which often play a role in coagulation. The next chapter starts with a description of the methodology of viscoelastic hemostatic assay, followed by a review of literature on the utilization of thrombelastography (TEG) or rotational thromboelastometry (ROTEM). Finally, the role of TEG and ROTEM to monitor or guide prophylaxis/treatment of thromboembolic events and the limitations of using them to detect hypercoagulability and identify patients with increased risks for thromboembolic complications are discussed. The final chapter of this book focuses on the use of fresh frozen plasma (FFP) in patients who are not actively bleeding, critically questions these assumptions in the context of pre-procedural administration of FFP, and also examines why carrying out high quality trails in this area has been so problematic.

Behcet's disease is a chronic relapsing inflammatory disease of unknown etiology involving multiple organs. Along with blindness, other bodily functions are greatly affected by this disease; conditions of the vascular, intestinal and central nervous systems are usually life threatening and require aggressive therapy. Recently, significant progress has been made in several areas of this disease. The introduction of biological agents (such as anti-TNF) have had a positive impact on the effectiveness of treatments. However, there are still a number of unmet needs in various fields of this disease. Despite this, there remains a number of unaddressed issues concerning the treatments of this disease. Several genes have been shown to be associated with this disease, in addition to HLA-B51. Abnormalities in several subsets of T helper cells have been implicated in the pathogenesis of Behcet's disease. Recent advances in the field of innate immunity have shed light on the new aspects of Behcet's disease; that is, this disease has both autoimmunity and autoinflammatory aspects. On the other hand, there are still controversies as to the diagnosis and treatment of recalcitrant manifestations of the disease. This publication highlights the most recent understanding of Behcet's disease and raises several unanswered questions in both basic to clinical treatment of this ailment. The contents of this book are primarily based on the most up-to-date results of research programs sponsored by the Japanese government as well as findings from Korean researchers. In particular, novel classification for neurological involvement (which has been discovered in Japan) led to the establishment of diagnostic criteria and treatment recommendation. Thus, this publication brings forth useful information for a variety of specialists who are involved in the management of this intractable disease.

Fanconi Anemia (FA), a rare genetic disease featuring excess cancer risk and chromosomal instability, has received growing interest after the discovery that one of the defective genes in FA is associated with high-impact diseases such as familial breast cancer and ovary cancers. A well-documented relationship has accumulated in several decades and up to recently, studies have linked FA with a redox imbalance (oxidative stress, OS) in FA cells. The toxicity mechanisms of crosslinking agents (mitomycin C and diepoxybutane) in FA cells, along with the roles of OS-related aldehydes and glutathione in FA and in other diseases, are critically discussed. Also discussed is the proinflammatory state in FA phenotype. Additionally, recent studies have discovered an impairment in the structure and function of mitochondria in FA cell lines, while mitochondrial dysfunction in FA patients has so far been an unexplored field warranting ad hoc investigations. This book provides the readers with up-to-date information and perspectives on Fanconi anemia and oxidative stress that may prompt further elucidation of this intriguing disease, along with working hypotheses in clinical research and patients' management.

Mantle cell lymphoma (MCL) is a subtype of Non-Hodgkin's Lymphoma (NHL) with varying clinical presentations, ranging from indolent disease to highly aggressive symptoms. MCL represents approximately 3-10% of non-Hodgkin lymphomas, and has increased in incidence over the past several decades. The majority of patients respond to initial therapy, but remission duration is typically short. This book discusses the clinical characteristics of mantle cell lymphoma, as well as its prevalence and examines several treatment options available.

This book discusses different aspects of neutropenia including drug-induced neutropenia, which is quite common, and neutropenia in chemotherapy-treated patients. The authors offer an all-encompassing approach to the pathobiology and treatment of neutropenic disorders. This book reviews current information concerning neutropenia. There have been significant advances in medicine; however, bacterial infections continue to contribute significantly to morbidity and mortality in patients with neutropenia particularly cancer patients who develop chemotherapy-induced neutropenia. In this setting, it is important to understand pathophysiology of neutropenia and have a logical approach in diagnosis and treatment of neutropenia. Gathering all this information in one book will be of immense benefit for healthcare workers including physicians, pharmacists, physician assistants, nurse practitioners and nurses and this in turn will help them improve the care of their patients. It will also be of benefit for pharmacists as a source of clinical and pharmacological knowledge. For researchers undertaking research in the field of hemato-oncology or pharmacology, this will be a useful starting point for reviewing the literature. The editors have tried to allow information in book chapters to show different aspects and various view points on related issues.

Decreased platelet counts can be due to a number of disease processes causing decreased platelet production, increased platelet destruction, or can be medication-induced. Health care providers have to differentiate inherited thrombocytopenias, primary immune thrombocytopenias (ITP), secondary immune thrombocytopenias, myelodysplastic syndromes with thrombocytopenia, bone marrow failure syndromes and non-immune thrombocytopenias. ITP is the most common cause of isolated thrombocytopenia. This book focuses on discussing ITP in adults; new treatment for ITP; thrombocytopenia during pregnancy; the different diagnosis of thrombocytopenia; heparin-induced thrombocytopenia; and thrombocytopenia in dengue.

Anaemia is defined as the decrease in haemoglobin from normal values either by loss of red blood cells or deficit in production or both. Haemoglobin is the major transporter of oxygen. The variation in haemoglobin is therefore a factor in determining the cardiac output. This book begins by discussing the effects anaemia has on heart diseases. The book then continues to discuss the influence of iron deficiency anaemia and recovery on oxidative/antioxidant status; influence of iron deficiency anaemia on bone metabolism; sickle cell anaemia; anaemia in myelodysplastic syndromes; transfusion in chronic anaemia; the prevalence, risk factors and management with a focus on chronic kidney disease; strategy for treating anaemia in chronic kidney disease patients from the standpoint of iron utility; and parasitic anaemia.

Myeloproliferative disorders are a group of clonal haematological neoplasms characterised by proliferation of one or more cells of myeloid lineage. They are the result of acquired mutations in the progenitor cell leading to hyper proliferation or neoplastic expansion of more mature forms of myeloid cells. Cells retain their functional ability with some degree of defects and also lead to suppression of normal stem cells. The most common type of Myeloproliferative Neoplasms (MPN) can broadly be classified into BCR ABL positive (Chronic Myelogenous Leukemia) and BCR ABL negative Disorders (Polycythemia Vera PV, Essential Thrombocytosis ET and Primary Myelofibrosis PMF). There are other rare types which have relatively low incidence like chronic neutrophilic leukemia, chronic eosinophilic leukemia, systemic mastocytosis and myeloproliferative neoplasms unclassifiable. These are the indolent type of haematological malignancies associated with marrow hypercellularity and organomegaly, with gradual progression to myelofibrosis or transformation to acute leukemias. During the dormant course of the BCR ABL negative MPN, they are more prone to thrombo-hemorrhagic complications and the treatment strategy is directed mostly to prevent complications. The past decade; therapies for BCR ABL positive disease (CML) have been a milestone achievement in keeping the disease in remission for many years, preventing major complications and halting the progression of the disease. This book discusses the classification, diagnosis and treatment of myeloproliferative diseases and provides insight on the symptoms and risk factors involved in the diseases.

Thalassemia is one of the most common genetic disorders worldwide and presents major public health and social challenges in areas of high incidence. The frequency of this disorder varies considerably with geographic locations and racial groups. Thalassemia refers to a group of inherited hemolytic anemia disorders that involve defects in the synthesis of hemoglobin - or -polypeptide chains. It leads to decreased hemoglobin production and hypochromic microcytic anemia associated with erythrocyte dysplasia and destruction. Homozygous -thalassemia (also known as thalassemia major, Cooley's anemia, or Mediterranean anemia) is associated with the most severe signs and symptoms. Thalassemia major (TM) is a life-threatening condition that commonly manifests during early infancy, after which progressive pallor, severe anemia, and failure to thrive are common. Children with TM often develop feeding problems, recurrent fever, bleeding tendencies (especially epistaxis), susceptibility to infection, pathologic fractures of long bones and vertebrae, endocrine abnormalities, splenomegaly, lack of sexual maturation, and growth retardation. This book discusses cures and treatments available for thalassemia, as well as the causes and the type of long-term health outcomes it may cause.

Inferior Vena Cava (IVC) filters have become an important part of deep venous thrombosis and pulmonary embolism treatment and prevention. With the advent of retrievable filters a new era of IVC instrumentation has been initiated. This book will begin to review the history of IVC filtration, indications for permanent and temporary filter use and filter use in special populations such as the pregnant patient or those with upper extremity venous thrombosis. The authors also review the technical aspects of filter placement and retrieval and discuss immediate and long-term complications from IVC filters. The book will also continue to discuss how acute spinal cord injury provides risks for developing deep vein thrombosis; and discuss the efficacy and safety of novel oral anticoagulants for venous thromboembolisms.

Plasmapheresis is a therapeutic tool used to treat a wide range of disease processes, and in which the priority aim is to ensure sufficient plasma exchange to reduce or eliminate symptoms resulting from the action of pathogenic elements vehiculised in the plasma. Scientific selective apheresis with principles of evidence-based medicine involves the application of one of the oldest therapeutic modalities (bleeding), widely used between the fourth century B.C. and the second industrial revolution. This book provides information on the use of plasmapheresis during pregnancy; therapeutic use of autologous plasma for the treatment of dry eye disease; and therapeutic plasma exchange in the neurological setting. It also discusses intravenous immunoglobulins. During the past decades intravenous immunoglobulins (IVIG) have gained more and more popularity for the treatment of a wide range of diseases and conditions. This treatment is extensively used in immune deficits, autoimmune thrombocytopenia, Kawasaki's disease, for the prevention of infectious complications due to hypogammaglobulinaemia secondary to myeloma, chronic lymphatic leukaemia and post-bone marrow and stem cell transplantation, in Guillain-Barre syndrome, etc. IV-Ig is obtained from the plasma of healthy blood donors and contains normal, polyclonal, polyspecific immunoglobins (Ig), mostly consisting of intact IgG. These antibodies are directed against non-self-antigens, self-antigens (natural autoantibodies), and other antibodies (idiotypic antibodies). The administration of intravenous immunoglobulin (IVIG) is generally safe and well tolerated. However, the treatment of autoimmune disorders usually requires high dose therapy (1-2 g/kg) that may result in a greater frequency of side effects and adverse events. This book discusses several topics including the clinical application of intravenous immunoglobulins in autoimmune mediated ocular inflammatory diseases; complications of intravenous immunoglobulin therapy; clinical uses; and side effects.

Thrombophilias can be defined as a group of inherited or acquired disorders that increase the risk of developing thrombosis. Venous thromboembolism (VT) is considered a multifactorial disease produced by a sum of risk factors that predispose to the thrombotic event. This predisposition includes genetic and acquired defects. Thrombosis can occur in any section of the venous system, but commonly manifests as deep vein thrombosis of the leg and pulmonary embolism. Major complications of venous thrombosis are a disabling post-thrombotic syndrome, pulmonary hypertension, and sudden death duo to a pulmonary embolism and therefore, it poses a burden on health economy. Venous thrombosis is a common clinical challenge for doctors of all disciplines, as it is a complex multicausal disease. This books discusses in further detail the many complications and risk factors caused by venous thrombosis.

von Willebrand disease (VWD) is an inherited bleeding disorder that is caused by deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII (FVIII) in the circulation. This book examines the need for more information on VWD prevalence and the relationship between low VWF levels of bleeding symptoms or risk and improving clinical and laboratory diagnostic tools.

This book presents topical research in the study of the types, treatments and health risks associated with hematomas. Topics discussed include the physiotherapeutic treatments of hematomas; cerebrospinal hematoma; infected hematomas; the etiology of pelvic hematomas; intracranial hematomas in pediatric patients; massive retroperitoneal hematoma following vaginal correction of vault prolapse and retropharyngeal hematomas.

Hemostasis (coagulation and fibrinolysis) is of importance in a broad range of medical disciplines. A high-quality hemostasis diagnosis enables a good therapy, which can be rather complicated without laboratory help. This new yearbook gathers important contributions in this field and presents them in a coherent and logical format.

This book presents topical research in the study of the kinetics, structure formation and disorders related to coagulation. Topics discussed include Brownian coagulation and diffusion-limited reactions; deregulation of coagulation during sepsis-induced disseminated intravascular coagulation; substrate induced coagulation (SIC) in aqueous and non-aqueous media for the preparation of advanced battery materials and neonatal coagulation problems. (Imprint: Nova)

Hemostasis (coagulation and fibrinolysis) is of importance in a broad range of medical disciplines. A high-quality hemostasis diagnosis enables a good therapy, which can be rather complicated without laboratory help. This yearbook gathers important contributions in this field and presents them in a coherent and logical format.

Hemostasis (coagulation and fibrinolysis) is of importance in a broad range of medical disciplines. A high-quality hemostasis diagnosis enables a good therapy, which can be rather complicated without laboratory help. This yearbook gathers important contributions in this field and presents them in a coherent and logical format.

In the human body, iron is present in all cells and has several vital functions: as a carrier of oxygen to the tissues from the lungs in the form of hemoglobin; as a transport medium for electrons within the cells in the form of cytochromes and as an integral part of enzyme reactions in various tissues. Too little iron can interfere with these vital functions and lead to morbidity and death. This book presents current research from across the globe in the study of iron deficiency, including iron deficiency anemia in pregnant women and in gastric bypass surgery patients; the metabolic adjustment under Fe deficiency in roots of dicotyledonous plants and strategies for the fortification of food with iron.