The only up-to-date definitive reference source onhemophilia This book is an invaluable resource that provides an overview ofall aspects of the care of patients with haemophilia. Covering how to assess both bleeding children and adults,Haemophilia A and B, molecular basis of the disease, the role offactors in coagulation, epidemiology, pharmacokinetics, andtreatment of inhibitors. There will also be a section onmusculoskeletal aspects of haemophilia as well as newerdevelopments such as gene therapy and rare bleedingdisorders. Textbook of Hemophilia is ideal for: Trainees and residents in hematology Hematologists in practice Specialists working in thrombosis and hemostasis as well astransfusion medicine Why Buy This Book? The only up-to-date definitive reference source onhemophilia Essential for all those managing hemophilia patients Detailed guidance on assessment, diagnosis, management andtreatment Advice for everyday clinical questions Edited by three of the world’s leading experts onhemophilia

Hemophilia and Von Willebrand Disease: Factor VIII and Von Willebrand Factor serves as a must-have reference on the important role these essential blood-clotting proteins play in research and clinical medicine. Clinicians and researchers face the daily challenge of staying current on the vast amounts of research that is now generated. The reference to Janus in the title refers to the two roles of the Factor VIII/Von Willebrand Factor Complex: initiation of coagulation and propagation of clot formation. The complex prevents bleeding in hemophilia and Von Willebrand disease but also augments arterial and venous thrombosis. Presents one source of information on Hemophilia and Von Willebrand Disease, as well as Factor VIII and Von Willebrand Factor, eliminating the search through hundreds of journal articles Combines the multi-disciplinary research that is generated from Factor VIII/Von Willebrand Factor – hematology, drug discovery, genetics, cell biology, and oncology Delves into unanswered questions and future directions of this important blood-clotting complex

Edited by an orthopaedic surgeon and a haematologist who are leading specialists in the treatment of haemophilia, Inhibitors in Patients with Haemophilia reviews the different haemostatic products and protocols for the control of bleeding and surgery in haemophilic patients with inhibitors. The book draws together in a single volume all of the clinical issues involved in the treatment of inhibitors from numerous specialists worldwide. It will be an invaluable resource for all those treating inhibitors in people with haemophilia.

This clinical casebook provides a concise, state-of-the-art review of pediatric bleeding disorders. Presented in a case-based format, each case presents a different variant of bleeding disorder, illustrates the etiology, pathology, genetics, diagnosis, and management of the disorder, and offers clinical pearls. Disorders covered include hemophilia A and B, rare factor deficiencies, von Willebrand disease, immune thrombocytopenia purpura, and platelet dysfunctions. Written by experts in the field, Pediatric Bleeding Disorders: A Clinical Casebook is a valuable resource for clinicians and practitioners treating patients with challenging coagulation issues.

The Textbook of Hemophilia has become a definitive resource for all those managing hemophilia patients. It covers all the common and rare bleeding disorders, both in terms of clinical management as well as the genetic, laboratory, financial and psychological aspects. This second edition covers all the latest developments in the field of hemophilia, with new chapters on: the genetic and molecular basis of inherited blood disorders how to manage adolescent and older patients emergency medicine and inherited blood disorders national hemophilia databases Drawing on the vast experience of the authors, the aim of this textbook remains the same - to improve the care of patients suffering from hemophilia. The book is full of detailed guidance and advice on everyday clinical questions making it invaluable to all trainee and practicing hematologists.

"For the blood is the life . . . . "(Deut. 12 :23) " . . . because the blood, in its value as life, makes atonement" (Lev. 17: 11) HemoPhilia is a rare disease, severe hemophilia rarer still, yet the written history of hemophilia extends back over a millennium and a half. In the ancient Middle East, blood and life were coupled. Blood was the primary substance necessary for life, given to God in sacrifice and forbidden as a food to mortals by Levitical law. Blood was essential for rites of purification and consecration. But the flow of blood during menstruation or parturition rendered a woman unclean. The circumcision of a male child required 33 days of "blood purification" by the mother. ' Circumcision, the visible reminder of the covenant of Abraham lijith Yahweh, was required of newborn Jewish males. It "connote(d) suitability for participation in what God is doing. "2 Hence, free and uncontrolled bleeding of the male child during circumcision, during the ratification of God's covenant, would be noted with awe and concern by those of the Jewish faith. It should not be surprising that the first genetic counseling offered to families with hemophilia is found in the Babylonian Talmud (compilation of Jewish law dated to about the third century AD) and concerns the necessity for circumcision in families with what we would now call hemophilia.

A succinct summary of the key principles and facts that guide the everyday practice of modern, clinical paediatric hematology. Covering all the information necessary for examinations in the topic, this book is ideal for postgraduates studying paediatric hematology, as well as for junior doctors in training.

Textbook of Hemophilia, 3rd edition Edited by Christine A. Lee, MA, MD, DSc, FRCP, FRCPath, FRCOG Emeritus Professor of Haemophilia, University of London, London, UK Erik E. Berntorp, MD, PhD Professor of Coagulation Medicine, Lund University Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden W. Keith Hoots, MD Director, Division of Blood Diseases and Resources, National Heart, Lung and Blood Institute National Institutes of Health, Bethesda, MD; Professor of Pediatrics and Internal Medicine, University of Texas Medical School at Houston, Houston, TX, USA Without doubt, Textbook of Hemophilia, 3rd edition is the definitive reference source on all aspects of haemophilia including diagnosis, management and treatment. Edited by three, world-renowned experts on haemophilia, this completely revised resource features chapters written by over 60 international contributors with international expertise in caring for haemophilia patients. Textbook of Hemophilia, 3rd edition Features eight new chapters, covering individualised dosing, vCJD and haemophilia, new drugs in the pipeline, and surgery in inhibitor patients Presents new developments, such as gene therapy Highlights controversial issues and provides advice for everyday clinical questions Represents essential reading for all healthcare professionals involved in the care of those with haemophilia Titles of related interest Hemophilia and Hemostasis: A Case-Based Approach to Management, 2nd Edition Ma, ISBN: 9780470659762 Current and Future Issues in Hemophilia Care Rodriguez-Merchan, ISBN: 9780470670576 www.wiley.com/go/hematology

Hemophilia A is an inherited X-linked bleeding disorder that occurs in male offspring of carrier females. In these individuals a mutation in the F8 gene, located on the X-chromosome, causes a deficiency of the factor VIII protein, clotting factor VIII. The worldwide prevalence of hemophilia is 1 in 5000 live male births, with one-third of the patients affected by severe hemophilia A and two-thirds of the patients have nonsevere disease. In patients with hemophilia A there is an impaired hemostasis due to a reduction of their factor VIII plasma level. This is associated with clinical symptoms of spontaneous bleeds in joints and muscles. An extremely severe and challenging complication of treatment with factor VIII concentrates in hemophilia A patients is the development of anti-factor VIII antibodies that inhibit (neutralize) the factor VIII activity, also referred to as inhibitors. This complication occurs in 25-30% of the severe hemophilia patients. Inhibitors seem to occur less frequently in nonsevere hemophilia A patients, although studies on inhibitor incidence in this group of patients are scarce. When a nonsevere hemophilia A patient develops an inhibitor, the phenotype of the disease may change dramatically. Nonsevere haemophilia A patients would normally only experience incidental bleeding episodes, but after inhibitor development the bleeding phenotype may aggravate dramatically, especially if the inhibitor reduces the endogenous factor VIII plasma levels. This is associated with an increased morbidity and mortality. This thesis focuses on the risk factors and treatment of inhibitors in nonsevere hemophilia A.