Primary Immunodeficiency Diseases

Definition, Diagnosis, and Management

  • Nima Rezaei
  • Asghar Aghamohammadi
  • Luigi D. Notarangelo

Table of contents

  1. Front Matter
    Pages I-XXVIII
  2. Nima Rezaei, Francisco A. Bonilla, Kathleen E. Sullivan, Esther de Vries, Jordan S. Orange
    Pages 1-38
  3. Françoise Le Deist, Despina Moshous, Steven J. Howe, Amit Nahum, Fotini D. Kavadas, Elana Lavine et al.
    Pages 39-95
  4. Asghar Aghamohmmadi, Vassilios Lougaris, Alessandro Plebani, Toshio Miyawaki, Anne Durandy, Lennart Hammarström
    Pages 97-130
  5. Uwe Wintergerst, Sergio D. Rosenzweig, Mario Abinun, Harry L. Malech, Steven M. Holland, Nima Rezaei
    Pages 131-166
  6. Carsten Speckmann, Jan Rohr, Stephan Ehl
    Pages 167-194
  7. Nima Parvaneh, Joachim Roesler, Steven M. Holland, Tim Niehues
    Pages 195-214
  8. Stefan Berg, Anders Fasth
    Pages 215-233
  9. Maryam Mahmoudi, Tom Eirik Mollnes, Taco W. Kuijpers, Dirk Roos
    Pages 235-249
  10. Mehdi Yeganeh, Eleonora Gambineri, Kamran Abolmaali, Banafshe Tamizifar, Teresa Español
    Pages 251-290
  11. Jeffrey E. Ming, E. Richard Stiehm
    Pages 291-314
  12. Hale Yarmohammadi, Charlotte Cunningham-Rundles
    Pages 315-334
  13. Back Matter
    Pages 335-358

About this book


Primary immunodeficiency diseases (PID) are a group of disorders involving defects in one or more components of the immune system, and are characterized by an increased incidence of infections, autoimmunity, and malignancies. Although PID seem to be rare, the number of patients diagnosed has increased in recent years, and more than 150 different forms of PID have been identified. Nevertheless, because of inadequate medical awareness, a significant number of patients with PID are either not recognized as having a PID or are not diagnosed as early as they should be. Such delays lead to a substantial increase in morbidity and mortality among affected individuals.
Our understanding of PID is improving rapidly, which will hopefully lead to more accurate diagnosis and efficient disease management. This book contains the most recent advances in the field, as well as a concise and structured review of previously identified PID. Although the book’s primary focus is on practical diagnosis and management, the pathophysiology of PID is also discussed.
This book is a comprehensive yet manageable resource for physicians and nurses wishing to learn more about PID, as well as a useful tool for both doctors-in-training and specialists in clinical decision-making and treatment planning.


Clinical Manifestations Definition Diagnosis Primary immunodeficiency diseases autoimmunity cell diseases immune system immunity immunodeficiency infection infections innate immunity pathophysiology physiology

Editors and affiliations

  • Nima Rezaei
    • 1
    • 2
  • Asghar Aghamohammadi
    • 3
  • Luigi D. Notarangelo
    • 4
  1. 1.Immunology, Asthma and Allergy Research Institute Children’s Medical Center HospitalTehran University of Medical SciencesTehranIran
  2. 2.Section of Infection, Inflammation and Immunity School of Medicine and Biomedical SciencesThe University of SheffieldSheffieldUK
  3. 3.Division of Allergy and Clinical Immunology Children’s Medical Center HospitalTehran University of Medical SciencesTehranIran
  4. 4.Division of Immunology Children’s HospitalHarvard Medical SchoolBostonUSA

Bibliographic information

  • DOI
  • Copyright Information Springer Berlin Heidelberg 2008
  • Publisher Name Springer, Berlin, Heidelberg
  • eBook Packages Medicine Medicine (R0)
  • Print ISBN 978-3-540-78537-8
  • Online ISBN 978-3-540-78936-9
  • Buy this book on publisher's site