Machine derived contents note: Part I: Overview -- 1. Genetically Determined Immunodeficiency Diseases: A Perspective, C.I. Edvard Smith, Hans D. Ochs, and Jennifer M. Puck -- 2. Genetic Principles and Technologies in the Study of Immune Disorders, Jennifer M. Puck and Robert L. Nussbaum -- 3. Mammalian Hematopoietic Development and Function, Gerald J. Sprangrude -- 4. T Cell Development, Rae S.M. Yeung, Joseph M. Penninger, and Tak W. Mak -- 5. Molecular Mechanisms Guiding B Cell Development, Antonius Rolink, Jan Andersson, Paolo Ghia, Ulf Grawunder, et al. -- 6. Signal Transduction by T and B Lymphocyte Antigen Receptors, Arthur Weiss, Anthony L. DeFranco -- 7. Phagocytic System, Kuender D. Yang, Harry R. Hill, Paul G. Quie -- Part II: Syndromes -- 8. X-linked Severe Combined Immunodeficiency, Jennifer M. Puck -- 9. Severe Combined Immune Deficiency Due to Defects of the JAK3 Tyrosine Kinase, Fabio Candotti and Luigi D. Notarangelo -- 10. Immunodeficiency Disease Due to Deficiency of Adenosine Deaminase (ADA), Rochelle Hirschhorn -- 11. Immunodeficiency Disease Due to Deficiency of Purine Nucleoside Phosphorylase, William R. Osborne and Hans D. Ochs -- 12. SCID Resulting From Mutations in the Gene Encoding the Protein Tyrosine Kinase ZAP-70, Melissa E.Elder and Arthur Weiss -- 13. Recombination Defects, Klaus Schwarz, Luigi Notarangelo, Eugenia Spanopoulou, and Anna Villa -- 14. Molecular Basis of Major Histocompatibility Complex Class II Deficiency, Walter Reith, Viktor Steimle, Barbara Lisowska-Grospierre, Alain Fischer, et al. -- 15. HLA Class I Deficiencies, Henri de la Salle, Lionel Donato, Jaques Zimmer, Alessandro Plebani, et al. -- 16. CD3 Deficiencies, Jose R. Reguieiro, A. Pacheco, D. Alvarez-Zapata, A. Corell, et al. -- 17. DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome, Deborah A. Driscoll and Kathleen E. Sullivan -- 18. Inherited Interferon-Y Receptor Deficiency, Jean-Laurent Casanova, Melanie Newport, Alain Fischer, and Michael Levin -- 19. X-Linked Lymphoproliferative Disease, Volker Schuster and Hans Wolfgang Kreth -- 20. CD40 Ligand and the Hyper IgM Syndrome, Narayanaswamy Remesh, Raif S. Geha, and Luigi D. Notarangelo -- 21. Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency, Lennart Hammerstrom and C.I. Edvard Smith -- 22. X-linked Agammaglobulinemia: A Disease of Btk Tyrosine Kinase, C.I. Edvard Smith and Owen N. Witte -- 23. Autosomal Recessive Agammaglobulinemia, Mary Ellen Conley -- 24. The Wiskott-Aldrich Syndrome, Hans D.Ochs and Fred S.Rosen -- 25. Ataxia-Telangiectasia, Martin F. Lavin and Yosef Shiloh -- 26. Ataxia-Telangiectasia Variants (Nijmegen Breakage Syndrome), Rolf-Dieter Wegner, Krystyna Chrzanowska, Karl Sperling, and Marcus Stumm -- 27. The Immunodeficiency of Bloom Syndrome, James German -- 28. Inherited Disorders with Autoimmunity and Defective Lymphocyte Regulation, Jennifer M. Puck, Alain Fischer, Stephen E. Strauss, Francoise Le Deist, et al. -- 29. Chronic Granulomatous Disease, Dirk Roos and John T. Curnutte -- 30. Cell Adhesion and Leukocyte Adhesion Defects, Amos Etzioni and John M. Harlan -- 31. Chediak-Higashi Syndrome, Richard A. Spritz -- 32. Genetically Determined Deficiencies of the Complement System, Kathleen E. Sullivan and Jerry A. Winkelstein -- Part III: Assessment and Treatment -- 33. Assessment of the Immune System, Helen M.Chapel and A. David B. Webster -- 34. Genetic Aspects of Primary Immunodeficiencies, Jennifer M. Puck -- 35. Immunodeficiency Mutation Databases, Mauno Vihinen, Heikki Lehvaslaiho, and Richard H.G. Cotton -- 36. Conventional Therapy of Primary Immunodeficiency Diseases, E. Richard Stiehm -- 37. Bone Marrow Transplantation for Primary Immunodeficiency Diseases, Rebecca H.Buckley and Alain Fischer -- 38. Gene Therapy, Fabio Candotti and R. Michael Blaese -- Index.