Primary immunodeficiency diseases, first recognized 60 years ago, are inherited disorders that affect human adaptive and innate immunity. In most cases, affected individuals experience recurrent infections, but they may also suffer from autoimmune diseases and malignancies. This third edition of Primary Immunodeficiency Diseases provides readers with the historic and scientific background, clinical presentations, immunologic characteristics, and the molecular/genetic underpinnings of this rapidly enlarging class of diseases. With up-to-date diagnostic tools and therapeutic options - from prophylactic anti-infective measures to hematopoietic stem cell transplantation and gene therapy - this volume will remain an authoritative resource on this increasingly important area.

Offering unique, comprehensive coverage of both basic science and clinical scenarios, Clinical Immunology: Principles and Practice, 6th Edition, brings you up to date with every aspect of this fast-changing field. It examines the molecular, cellular, and immunologic bases of immunologic diseases and their broader systemic implications; it also includes complete coverage of common and uncommon immunologic disorders. Updated with all the latest immunologic research and clinical implications, including breakthrough immunotherapies and molecular-based treatment protocols, this fully revised edition provides authoritative guidance from some of the most respected global leaders in immunology in one complete, well-illustrated volume. Includes extensive revisions that reflect rapidly expanding research and clinical advances, including breakthrough drug and immunotherapies such as immune checkpoint inhibitors, immunotherapies for cancer, precision medicine, and transfusion medicine. Contains new chapters on COVID-19, immune responses, and the role of the immune system; immunoregulatory deficiencies; immune checkpoints; CAR T cells, including new cellular-based immunotherapy; gene therapy, including CRISPR and gene selection; and a clinically focused chapter on asthma. Provides new genetics content focused on data applications. Addresses notable advances in key areas such as the importance of the microbiota to normal immune system development and to the pathogenesis of immunologic and inflammatory diseases; relationships between the innate and adaptive immune systems; progress in rapid and cost-effective genomics; cell signaling pathways and the structure of cell-surface molecules; and many more. Covers hot topics such as the role of genetics and genomics in immune response and immunologic disease, atherosclerosis, recurrent fever syndromes, aging and deficiencies of innate immunity, the role of microbiota in normal immune system development and in the pathogenesis of immunologic and inflammatory diseases, and novel therapeutics. Features a user-friendly format with color-coded boxes highlighting critical information on Key Concepts, Clinical Pearls, Clinical Relevance, and Therapeutic Principles. Summarizes promising research and development anticipated over the next 5-10 years with "On the Horizon" boxes and discussions of translational research. An eBook version is included with purchase. The eBook allows you to access all of the text, figures and references, with the ability to search, customize your content, make notes and highlights, and have content read aloud.

The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders - disorders featuring an increased susceptibility to infections and, in, certain conditions, an increased rate of malignancies and autoimmune disorders. Since the publication of the first edition, a flurry of new disease entities has been defined and new treatment regimens have been introduced, the most spectacular being successful treatment by gene therapy for two genotypes of combined immunodeficiency. The first edition marked a historic turning point in the field of immunodeficiencies, demonstrating that many of the disorders of the immune system could be understood at a molecular level. This new edition can proudly document the tremendous pace of progress in dissecting the complex immunologic networks responsible for protecting individuals from these disorders.