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

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.

Researchers from the National Institutes of Health in Bethesda, Maryland, along with a few other contributors, explore ten disorders that may be linked only by resulting in a lowered blood count that can be traced to the failure of the bone marrow. They are acquired aplastic anemia, Fanconi's anemia, myelodysplastic syndromes, paroxysmal nocturnal hemoglobinuria, myelofibrosis, pure red cell aplasia, agranulocytosis, acquired amegakaryocytic thrombocytopenic purpura, bone marrow failure related to human immunodeficiency virus, and T cell large granular lymphocyte lymphoproliferative disorder. The text is supported by 13 color plates. The index is particularly detailed. Annotation copyrighted by Book News, Inc., Portland, OR

This book focuses on hematopoietic and lymphoid neoplasms that initially present as peripheral blood abnormalities, with either cytopenias or elevated peripheral blood counts, as well as non-neoplastic conditions that may raise concern for a hematologic malignancy. The scope of the book includes myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPN), mixed myelodysplastic/myeloproliferative neoplasms (MDS/MPN), as well as lymphomas and lymphoid leukemias that typically present initially with peripheral blood abnormalities. Within each category, a comprehensive list of differential diagnoses is discussed. For each disease entity, the reader is updated with new molecular genetic data, biomarkers, and recent applications of immunophenotyping, and how to incorporate the new information in disease diagnosis and classifications is illustrated, including the use of diagnostic algorithms where appropriate. The book employs the revised WHO Classification of Hematopoietic Neoplasms for all disease entities. Diagnosis of Blood and Bone Marrow Disorders will serve as a very useful resource for pathologists, pathologists in training, hematologists and medical technologists who are involved in the clinical work-up of patients with bone marrow and blood neoplasms. It will provide a practical and concise yet comprehensive review.

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.

This issue of Hematology/Oncology Clinics, edited by Dr. Colin A. Sieff, will focus on Bone Marrow Failure. Topics include, but are not limited to, Acquired and Inherited Bone Marrow Failure; Kickapoo Joy Juice and Somatic Mutations in the Pathogenesis of AA; Somatic Mutations in Aplastic Anemia; Recent Advances and Long-term Results of Medical Treatment of AA; Upfront Matched Unrelated Donor Transplantation in AA; Significance of Clonal Mutations in the Diagnosis and Management of Myelodysplastic Syndrome; Alternate (Haploidental) Donor Transplantation in AA; Management of Diamond Blackfan Anemia and Prospects for Novel Treatment; MDS, AML and Cancer Surveillance in Fanconi Anemia; Diagnosis, Treatment and Molecular Pathology of Shwachman Diamond Syndrome; Clinical Implications of Clonal Hematopoiesis in Dyskeratosis congenita; and Germline GATA2 Mutations and Bone Marrow Failure.

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.

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.

Adolescent females experience a variety of blood disorders that are often unique to this patient population. As they go through puberty, they are uniquely poised to encounter various bleeding and thrombotic disorders once they attain menarche, start to have menstrual bleeding, and require hormonal therapy. This may in turn lead to other medical conditions, such as anemia and iron deficiency. Pregnancy encountered by some adolescents can pose hematologic challenges specifically in regards to bleeding and thrombotic disorders. In addition, adolescent females are at risk to develop immune mediated hematologic disorders, such as immune thrombocytopenia, auto-immune hemolytic anemia, and thrombotic thrombocytopenic purpura, as well as vitamin deficiencies due to pernicious anemia. Sickle cell disease, thalassemia and bone marrow failure disorders in the adolescent female poses unique challenges that need to be addressed with special care and attention. Knowledge about these various blood disorders in adolescent females is crucial for the treating physician in order to accurately diagnose and optimally manage these teenagers. Otherwise, it can affect their overall health, causing hematologic and gynecologic issues, poor quality of life, neurocognitive impairments, and poor psycho-social development, all of which can lead to various complications immediately and into adulthood. This book provides a comprehensive, state-of-the art overview of blood disorders in female adolescents. The text presents new data about bleeding disorders that affect the female adolescent, including bleeding disorders, thromboembolism, thrombophilia, anemia, sickle cell disease and thalassemia, disorders od hemostasis and thrombosis in pregnancy, immune hematology and bone marrow failure disorders; reviews our current understanding of these disorders; outlines recent research findings; and spotlights multi-disciplinary approaches, evaluation and treatment modalities to combat these blood disorders. Written by experts in the field, Hematology in the Adolescent Female is a valuable resource for clinicians and practitioners who treat and manage female adolescents with blood disorders.