© 2019

Humoral Primary Immunodeficiencies

  • Mario Milco D'Elios
  • Marta Rizzi


  • Describes basic science discoveries and the latest clinical advances

  • Structured in accordance with the most recent classification of humoral PIDs

  • Covers updates in the B cell immunological synapse

  • Provides in-depth descriptions of novel humoral PID genes and related clinical applications

  • Documents the different clinical phenotypes of humoral PIDs


Part of the Rare Diseases of the Immune System book series (RDIS)

Table of contents

  1. Front Matter
    Pages i-x
  2. Laura Patrussi, Nagaja Capitani, Mario Milco D’Elios, Cosima T. Baldari
    Pages 1-20
  3. Arianna Troilo, Nagaja Capitani, Laura Patrussi, Cosima T. Baldari, Mario Milco D’Elios
    Pages 21-34
  4. Ulrich Salzer
    Pages 35-55
  5. Eduardo López-Granados
    Pages 57-66
  6. Vasssilios Lougaris, Alessandro Plebani
    Pages 67-76
  7. Ulrich Salzer
    Pages 77-82
  8. Menno C. van Zelm, Ismail Reisli
    Pages 83-95
  9. Jens Thiel
    Pages 97-99
  10. Astrid Bergbreiter, Ulrich Salzer
    Pages 101-112
  11. Laura Gámez-Díaz
    Pages 113-129
  12. Cristian R. Smulski, Patricia Odermatt, Hermann Eibel
    Pages 131-147
  13. Ulrich Salzer
    Pages 149-152
  14. Shancy P. Jacob, Julie E. Feusier, Karin Chen
    Pages 153-165
  15. Giuliana Roselli, Marinos Kallikourdis, Antonella Viola
    Pages 167-177
  16. Mirjam van der Burg, Andrew R. Gennery, Qiang Pan-Hammarström
    Pages 179-199
  17. K. Sofia Appelberg, Hassan Abolhassani, Lennart Hammarström
    Pages 201-215
  18. Isabella Quinti, Cinzia Milito, Rossella Carello, Federica Pulvirenti
    Pages 235-252

About this book


This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed. The authors are all recognized experts from Europe, Australia, and the United States. Humoral Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students.


B-cell Antibodies Gammaglobulines Common Variable Immune Deficiency Clinical Phenotypes PID genes Autoimmunity Infections Lymphoma Cancer Tumor

Editors and affiliations

  • Mario Milco D'Elios
    • 1
  • Marta Rizzi
    • 2
  1. 1.Careggi HospitalDepartment of Clinical and Experimental Medicine, University of FlorenceFirenzeItaly
  2. 2.University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic ImmunodeficiencyFreiburgGermany

About the editors

Mario M. D'Elios, MD, PhD, Specialist in Clinical Immunology & Allergy, Professor of Internal Medicine, Group leader, chief of the Nanomedicine and Immunology unit at the University of Florence, Italy. Expert in mucosal immunology, primary immunodeficiencies and infectious diseases.

Marta Rizzi, MD, PhD, Specialist in Allergy and Clinical Immunology, Group leader, Margarete von Wrangell fellow, at the Department of Rheumatology and Clinical Immunology /Centre of Chronic Immunodeficiency, University Medical Centre Freiburg, Germany, Expert in human B lymphocytes development, primary immunodeficiency and immune dysregulation.

Bibliographic information

  • Book Title Humoral Primary Immunodeficiencies
  • Editors Mario Milco D'Elios
    Marta Rizzi
  • Series Title Rare Diseases of the Immune System
  • Series Abbreviated Title Rare Diseases of Immune System
  • DOI
  • Copyright Information Springer Nature Switzerland AG 2019
  • Publisher Name Springer, Cham
  • eBook Packages Medicine Medicine (R0)
  • Hardcover ISBN 978-3-319-91784-9
  • eBook ISBN 978-3-319-91785-6
  • Series ISSN 2282-6505
  • Series E-ISSN 2283-6403
  • Edition Number 1
  • Number of Pages X, 387
  • Number of Illustrations 0 b/w illustrations, 34 illustrations in colour
  • Topics Rheumatology
    Infectious Diseases
  • Buy this book on publisher's site
Industry Sectors
Health & Hospitals
Internal Medicine & Dermatology