Publisher's Synopsis
The primary immunodeficiency disorders have been known for the past seven decades; however, immunological defects were described at the cellular level and the therapies were limited to the use of gamma globulin, antibiotics, and bone marrow transplantation. During the past decade, rapid progress has been made in the understanding of the basic concept of immune response at the biochemical and molecular levels. This new information has been instrumental in understanding the defects of many primary immunodeficiency disorders at the molecular level. As a result, innovative techniques are now available to diagnose them at a very early stage and to detect their carrier states. Novel therapeutic approaches, including gene therapy and use of biological response modifiers, have emerged. This book reviews these rapidly developing concepts in primary immunodeficiency disorders.;The book is divided into three major sections. Section 1 addresses basic mechanisms in immune response. This includes development of human antibody repertoire, pathways of human B cell growth and differentiation development of T cell repertoire, signalling mechanisms in T cell activation and T cell tolerance, and the role of adhesion molecules in neutrophil-endothelial cell interactions. Also included is a review on substituted quanine ribonucleoside compounds as B cell activators and their potential future use in the therapy of antibody deficiency syndromes. Section 2 deals with the genetic and molecular basis of primary immunodeficiency disorders. The genetic and molecular basis of Leu-CAM (adhesion molecule) deficiency is reviewed.;Included in Section 3 are the advances in the area of therapy for primary immunodeficiency disorders. The current status of peripheral stem cell transplantation, PEG-ADA, and gene therapy in severe combined immunodeficiency have been reviewed. The use of biological response modifiers including interferons in chronic granulomatous disease and IL-2 in common variable immunodeficiency, has been discussed. A chapter is devoted to the use of colony stimulating factor and its haematological and clinical effects in congenital neutropenia, cyclic neutropenia, and acquired idiopathic neutropenia. The uses, guidelines, and mechanisms of action of intravenous immune globulin are reviewed.
