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  • © 2019

Humoral Primary Immunodeficiencies

  • 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 book series: Rare Diseases of the Immune System (RDIS)

Buying options

eBook USD 109.00
Price excludes VAT (USA)
  • ISBN: 978-3-319-91785-6
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
Hardcover Book USD 149.99
Price excludes VAT (USA)

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Table of contents (26 chapters)

  1. Front Matter

    Pages i-x
  2. The B-Side of the Immune Response

    • Laura Patrussi, Nagaja Capitani, Mario Milco D’Elios, Cosima T. Baldari
    Pages 1-20
  3. Mucosal B Cells

    • Arianna Troilo, Nagaja Capitani, Laura Patrussi, Cosima T. Baldari, Mario Milco D’Elios
    Pages 21-34
  4. CVID

    • Ulrich Salzer
    Pages 35-55
  5. Late-Onset Combined Immunodeficiencies (LOCID)

    • Eduardo López-Granados
    Pages 57-66
  6. Genetics of CVID

    • Vasssilios Lougaris, Alessandro Plebani
    Pages 67-76
  7. ICOS Deficiency

    • Ulrich Salzer
    Pages 77-82
  8. CD19 Deficiency due to Genetic Defects in the CD19 and CD81 Genes

    • Menno C. van Zelm, Ismail Reisli
    Pages 83-95
  9. Genetic CD21 Deficiency

    • Jens Thiel
    Pages 97-99
  10. TACI Deficiency

    • Astrid Bergbreiter, Ulrich Salzer
    Pages 101-112
  11. LRBA Deficiency

    • Laura Gámez-Díaz
    Pages 113-129
  12. BAFF Receptor Deficiency

    • Cristian R. Smulski, Patricia Odermatt, Hermann Eibel
    Pages 131-147
  13. TWEAK Deficiency

    • Ulrich Salzer
    Pages 149-152
  14. NFKB2 Defects

    • Shancy P. Jacob, Julie E. Feusier, Karin Chen
    Pages 153-165
  15. The WHIM Syndrome

    • Giuliana Roselli, Marinos Kallikourdis, Antonella Viola
    Pages 167-177
  16. Class-Switch Recombination Defects

    • Mirjam van der Burg, Andrew R. Gennery, Qiang Pan-Hammarström
    Pages 179-199
  17. Selective IgA Deficiency

    • K. Sofia Appelberg, Hassan Abolhassani, Lennart Hammarström
    Pages 201-215
  18. Differential Diagnosis in Hypogammaglobulinemia

    • 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.

Keywords

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

Editors and Affiliations

  • Careggi Hospital, Department of Clinical and Experimental Medicine, University of Florence, Firenze, Italy

    Mario Milco D'Elios

  • University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency, Freiburg, Germany

    Marta Rizzi

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

Buying options

eBook USD 109.00
Price excludes VAT (USA)
  • ISBN: 978-3-319-91785-6
  • Instant PDF download
  • Readable on all devices
  • Own it forever
  • Exclusive offer for individuals only
  • Tax calculation will be finalised during checkout
Hardcover Book USD 149.99
Price excludes VAT (USA)