Primary Immunodeficiency Disorders: A Historic and Scientific Perspective

Primary Immunodeficiency Disorders: A Historic and Scientific Perspective provides a complete historical context that is crucial for students and researchers concerned with primary immunodeficiency. When researchers have a poor understanding of the way we arrived where we are in research, they can miss important points about a disease, or miss out on how to approach new diseases. This historical knowledge of research can assist greatly by showing how it was done in the past, demonstrating the successes and failures, so that it can be done better in the future.

This book provides an understanding of the process going from clinical problem to lab and back to the clinic, based on historical experiences. Its chapters proceed from the discovery of the T and B cell lineages through the first BMT for immunodeficiency disorder; lab investigation and gene therapy for PID; the discovery of the gene for AT and its function; understanding cytokine defects; and many other stops along the way.

Facilitates communication among physicians and other investigators concerned with immunological and inflammatory diseasesSummarizes for the first time all the known facts from 60 years of primary immunodeficiency research, and teaches how an important field in medicine was establishedProvides stimulating discussions on developing new medical therapies Highlights the importance of studying humans to understand mechanisms of disease that affect humans

Foreword
Introduction
1. Immunity: From Serendipitous Observations To Science-Based Speciality
2. The Discovery Of The T And B Cell Compartments
3. Evolution Of The Definition Of Primary Immunodeficiencies
4. From Immunodeficiency To Autoimmunity
5. Immunological Tests – From The Microscope To Whole Genome Analysis
6. Primary Immunodeficiency In The Developing Countries
7. From A Sad Family History To A Worldwide Recognition
8. Finally Found: The Ataxia-Telangiectasia Gene and Its Function
9. Wiskott-Aldrich Syndrome: From A Fatal Hematologic Disorder To A Curable Immunodeficiency
10.Neutropenia – More Genetic Defects Than Ever Expected
11.B Cell Defects - From X Linked To Autosomal Recessive Agammaglobulinemia
12.The Discovery Of The Familial Hemophagocytosis Syndromes
13.Chronic Granulomatous Disease – From A Fatal Disease To A Curable One
14.Severe Combined Immunodeficiency - From Discovery To Newborn Screening
15.Cytokine Defects – How They Cause Severe Combined Immunodeficiency
16.The Hyper IgM Syndromes – A Long List Of Genes And Years Of Discovery
17.Unraveling The Complement System and Its Mechanism Of Action
18.The DiGeorge Syndrome: A Serendipitous Discovery
19.The Many Faces Of The Hyper IgE Syndrome
20.ADA Deficiency – The First Described Genetic Defect Causing PID
21.The Leukocyte Adhesion Deficiency Story
22.How Common Variable Immune Deficiency Has Changed Over Six Decades
23.From Subcutaneous To Intravenous Immunoglobulin And Back
24.History of Hematopoietic Stem Cell Transplantation
25.David's Story
26.How Primary Immunodeficiencies Have Made Gene Therapy A Reality