Table of Contents

1: C.I. Edward Smith, Hans D. Ochs, and Jennifer M. Puck: Genetically Determined Immunodeficiency Diseases: A Perspective
2: Jennifer M. Puck and Robert L. Nussbaum: Genetic Principles and Technologies in the Study of Immune Disorders
3: Gerald J. Sprangrude: Mammalian Hematopoietic Development and Function
4: Rae S. M. Yeung, Josef M. Penninger, and Tak W. Mak: T-Cell Development
5: Antonius Rolink, Jan Andersson, Ulf Grawunder, and Fritz Melchers: Molecular Mechanisms Guiding B-Cell Development
6: Neetu Gupta, Anthony L. DeFranco, and Arthur Weiss: Signal Transduction by T and B Lymphocyte Antigen Receptors
7: Sirpa Jalkanen and Marko Salmi: Regulation of Lymphocyte Responses, Cell Trafficking and Lymphoid Organ Development
8: Kuender D. Yang, Anthony Segal, Harry R. Hill: The Phagocytic System
Part II Syndromes:
9: Jennifer M. Puck: X-linked Severe Combined Immunodeficiency
10: Fabio Candotti and Luigi Notarangelo: Autosomal Recessive SCID Due to Defects of Cytokine Signaling Pathways
11: Anna Villa, Klaus Schwarz, and , J.P. Villartay: Recombination Defects
12: Rochelle Hirschhorn, Fabio Candotti: Immunodeficiency Due to Defects of Purine Metabolism
13: Markku Heikinheimo and Talal Chatila: CD45 Deficiency
14: Melissa E. Elder: SCID Due to Defects in T-Cell-Receptor-Associated Protein Kinases
15: Chaim M. Roifman: Human Interleukin-2 Receptor Alpha Deficiency
16: Jose R. Regueiro Teresa Espanol et al.: CD3 and CD8 Deficiencies
17: Walter Reith, Barbara Lisowska-Grospierre, and Alain Fischer: Molecular Basis of Major Histocompatibility Complex Class II Deficiency
18: Henri de la Salle, Lionel Donato, Daniel Hanau, and Marie-Marthe Tongio: Peptide Transporter Defects in HLA Class I Deficiency
19: Raif S. Geha, Alessandro, and Luigi Notarangelo: CD40, CD40 Ligand, and theHyper-IgM Syndrome
20: Anne Durandy, Patrick Revy, and Alain Fischer: Autosomal Hyper-IgM Syndromes Caused by an Intrinsic B-cell Defect
21: C.I. Edvard Smith and Owen N. Witte: X-linked Agammaglobulinemia: A Defect of Btk Tyrosine Kinase
22: Mary Ellen Conley: Autosomal Recessive Agammaglobulinemia
23: Lennart Hammarstrm and C.I. Edvard Smith: Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency
24: Jennifer M. Puck, Stephen E. Straus, Francoise Le Deist, Frederic Rieux-Laucat: Defects in Apoptosis with Autoimmune Manifestations
25: Leena Peltonen, Maria Halonen, and Jaakko Perheentupa: Autoimmune Polyendocrinopathy, Candidiasis and Ectodermal Dystrophy (APECED)
26: Hans D. Ochs, Mary E. Brunkow, and Steve Siegler: Immune Dysregulation, Polyendocrinopathy, Enteropathy; X-linked (IPEX)
27: Daniel Kastner, Susannah Brydges, and Keith M. Hull: Periodic Fever Syndromes
28: Melanie Newport, Steve Holland, Michael Levin, and Jean-Laurent Casanova: Inherited Disorders of the Interleukin-12/23-Interferon Gamma Axis
29: Martin F. Lavin and Yosef Shiloh: Ataxia-Telangiectasia
30: Karl Sperling, Markus Stumm, James German, Fanconi expert (e.g. Johnson Liu/French woman/Dutch): DNA Breakage and Repair Associated Syndromes Other Than Ataxia-Telangiectasia
31: Hans D. Ochs, Fred S. Rosen: The Wiskott-Aldrich Syndrome
32: Volker Schuster and Cox Terhorst: X-linked Lymphoproliferative Disease (XLP) due to Defects of SH2D1A
33: Deborah A. Driscoll and Kathleen E. Sullivan: DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome
34: Bodo Grimbacher and Stevn M. Holland: Hyper IgE Recurrent Infection Syndromes
35: Scott Hansen, Corry Weemaes, and Cisca Wijmenga: Immunodeficiency with Centromere Instability and Facial Anomalies
36: Mario Abinun, Ilkka Kaitila and Jean-Laurent Casanova: Immunodeficiencies with Associated Manifestations of Skin, Hair, Teeth, and Skeleton
37: Dirk Roos, Taco W. Kuijpers, and John T. Curnutte: Chronic Granulomatous Disease
38: Amos Etzioni and John M. Harlan: Cell Adhesion and Leukocyte Adhesion Defects
39: David Dale and Andrew Aprikyan: Cyclic and Congenital Neutropenia Due to Defects in Neutrophil Elastase
40: Richard A. Spritz: Chediak-Higashi Syndrome
41: Genevive de Saint Basile: Inherited Hemophagocytic Syndromes
42: Kathleen E. Sullivan and Jerry A. Winkelstein: Genetically Determined Deficiencies of the Complement System
Part III Assessment and Treatment:
43: Helen M. Chapel, Siraj Misbah, and David Webster: Assessment of the Immune System
44: Jennifer M. Puck: Genetic Aspects of Primary Immunodeficiencies
45: Jouni Valiaho, Crina Samarghitean, Hilkka Piirila, Marianne Pusa, and Mauno Vihinen: Immunodeficiency Information Systems
46: E. Richard Stiehm and Helen M. Chapel: Conventional Therapy of Primary Immunodeficiency Diseases
47: Rebecca H. Buckley and Alain Fischer: Bone Marrow Transplantation for Primary Immunodeficiency Diseases
48: Fabio Candotti and Alain Fischer: Gene Therapy

Reviews

"This latest edition of Primary Immunodeficiency Diseases emphasized the molecular bases of these disorders, but there are also thorough discussions of their clinical aspects, and these will make the book an important addition to the library of any physician who treats patietns with primary immunodeficiencies."--New England Journal of Medicine
"This latest edition of Primary Immunodeficiency Diseases emphasized the molecular bases of these disorders, but there are also thorough discussions of their clinical aspects, and these will make the book an important addition to the library of any physician who treats patietns with primary immunodeficiencies."--New England Journal of Medicine