Aplastic Anemia

Comprehensive and up-to-date clinical reference, with an emphasis on treatment.

Research on aplastic anaemia has until recently been limited to clinical description, morphology and epidemiology. New methods to culture haemopoietic cells, and advances in our knowledge of proliferation and differentiation in the haemopoietic cell system .opened a new area of scientific interest for this "prototype" of haemopoietic failure. In addition, bone marrow transplantation became not only a clinical method of treatment, but also a source of data useful for the discussion of pathophysiological models of aplastic anaemia. This situation prompted us to arrange an international con ference on aplastic anaemia, with particular emphasis on its patho physiology and the rationals of the current therapeutic approaches. This conference was held at Schloss Reisensburg from July 20-22, 1978 with the participation of both experimental and clinical scientists active in this field or in related areas of research. The proceedings of the symposion reflect the present knowledge as well as the many new questions which arose from the discussions. The editors are gratefully indebted to the participants of this meeting, to Gerlinde Trogele and all the co-workers of the Uni versity of Ulm engaged in preparation of this symposium and of this volume, and last not least to all sponsors who provided the financial basis for this scientific event.

Handbook of Benign Hematology is a practical guide to the diagnosis and management of benign hematologic conditions. The book begins with a chapter on normal hematopoiesis and follows with chapters devoted to groups of blood disorders and syndromes including neutrophil disorders, nonmalignant myeloid disorders, bone marrow failure syndromes, myeloproliferative disorders, anemias, iron metabolism disorders, platelet disorders, hemostasis and coagulation defects, and thrombosis. Each disorder subtype covered features a clinical case, an introduction to the condition, details on diagnosis including applicable criteria and lab work needed, key diagnostic dilemmas, prognosis, treatment options, details on clinical trials and emerging clinical strategies, and bulleted key points to highlight clinical pearls and common pitfalls. The final chapters provide best practices for transfusion medicine and a guide to pharmacologic agents and their uses in clinical practice for adult and pediatric patients. The handbook is filled with tables and illustrations which highlight FDA-approved drug information, clinical trials data, hematopathologic characteristics of different disorders, important management criteria and more, making it the ideal handbook for those in practice or for review. The Editors and chapter authors are experienced academic practitioners in the fields of adult and pediatric hematology, pathology, blood banking, and pharmacology. Emphasizing best practices for patient management, this handbook is essential for oncologists, hematologists, trainees, and other practitioners who regularly or increasingly receive referrals to diagnose and treat adults or children with nonmalignant hematologic conditions. Key Features: Includes dozens of clinical cases covering all nonmalignant blood disorders Emphasizes patient management and best practices for disorders seen in adults and children Contains over 30 color images and numerous tables for quick reference Presents important details of all pharmacologic agents used to treat or manage hematologic disorders and their complications Purchase includes access to the ebook for use on most mobile devices or computers

This Open Access edition of the European Society for Blood and Marrow Transplantation (EBMT) handbook addresses the latest developments and innovations in hematopoietic stem cell transplantation and cellular therapy. Consisting of 93 chapters, it has been written by 175 leading experts in the field. Discussing all types of stem cell and bone marrow transplantation, including haplo-identical stem cell and cord blood transplantation, it also covers the indications for transplantation, the management of early and late complications as well as the new and rapidly evolving field of cellular therapies. This book provides an unparalleled description of current practices to enhance readers' knowledge and practice skills. This work was published by Saint Philip Street Press pursuant to a Creative Commons license permitting commercial use. All rights not granted by the work's license are retained by the author or authors.

During the past decade, there have been numerous direct and indirect scientific contributions to both the etiology and therapy of aplastic anemia and related bone marrow failure syndromes. Clinical observations, such as autologous bone marrow recovery after conditioning with immunosup pressive agents for bone marrow transplantation; failure to achieve en graftment in some identical twins without prior immunosuppressive ther apy; and hematologic response to immunosuppressive agents, have led to the concept of immune-mediated etiology of acquired aplastic anemia. Such a concept was further strengthened by laboratory findings, implicat ing the role of activated cytotoxic T lymphocytes and abnormal produc tion of inhibitory lymphokines. The immunologic mechanisms may also apply to the idiosyncratic bone marrow aplasias associated with drugs, toxic chemicals, and viruses. These agents may alter normal cellular recog nition sites by interacting with cellular components and result in loss of self tolerance. Immunologic mechanisms have long been advocated in many other organ failures, and the hemopoietic organ is no exception. It is of interest that parallel clinical and laboratory investigations in juvenile diabetes mellitus type I and in rodent models of this disease have yielded results compatible with the same pathogenic mechanisms. The infiltration of pancreatic islets by activated T lymphocytes, functional and morphological alterations of islet cells upon incubation with lymphokines such as gamma interferon and tumor necrosis factor, and clinical response to cyclosporine are a few examples.

Congenital and Acquired Bone Marrow Failure is a comprehensive guide to congenital and acquired bone marrow failure in adult and pediatric patients. Chapters are divided into two sections, acquired aplastic anemia and inherited bone marrow failure syndromes. Content ranges from the basic, to the translational, and from the epidemiology of acquired aplastic anemia and telomere biology, to the management, treatment, and supportive care of pediatric, adult, and geriatric patients. Contributors are world leading experts in the field of bone marrow failure. The book is required reading for residents, fellows, clinicians, and researchers across hematology, oncology, pathology, bone marrow transplantation, pediatrics, and internal medicine. Provides an overview of all congenital and acquired bone marrow failure syndromes Focuses on the molecular pathogenesis, clinical manifestation and diagnosis, laboratory features, and treatment of each disease within the syndromes Features the area of supportive care which is a topic of great interest to infectious disease physicians and those involved in transfusion services

During the past decade, there have been numerous direct and indirect scientific contributions to both the etiology and therapy of aplastic anemia and related bone marrow failure syndromes. Clinical observations, such as autologous bone marrow recovery after conditioning with immunosup pressive agents for bone marrow transplantation; failure to achieve en graftment in some identical twins without prior immunosuppressive ther apy; and hematologic response to immunosuppressive agents, have led to the concept of immune-mediated etiology of acquired aplastic anemia. Such a concept was further strengthened by laboratory findings, implicat ing the role of activated cytotoxic T lymphocytes and abnormal produc tion of inhibitory lymphokines. The immunologic mechanisms may also apply to the idiosyncratic bone marrow aplasias associated with drugs, toxic chemicals, and viruses. These agents may alter normal cellular recog nition sites by interacting with cellular components and result in loss of self tolerance. Immunologic mechanisms have long been advocated in many other organ failures, and the hemopoietic organ is no exception. It is of interest that parallel clinical and laboratory investigations in juvenile diabetes mellitus type I and in rodent models of this disease have yielded results compatible with the same pathogenic mechanisms. The infiltration of pancreatic islets by activated T lymphocytes, functional and morphological alterations of islet cells upon incubation with lymphokines such as gamma interferon and tumor necrosis factor, and clinical response to cyclosporine are a few examples.

This book describes the findings of a rigorous and comprehensive epidemiological investigation designed to provide reliable quantitative data on the drug etiology of agranulocytosis and aplastic anemia.