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Rare Diseases of the Immune System

Agammaglobulinemia

Editors: Plebani, Alessandro, Lougaris, Vassilios (Eds.)

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  • Provides a comprehensive and detailed overview of a rare form of primary immunodeficiency 
  • Explains the genetic basis of the disease, with an overview of animal models
  • Describes advanced and experimental therapeutic approaches
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eBook $109.00
price for USA in USD
  • ISBN 978-3-319-22714-6
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  • Immediate eBook download after purchase
Hardcover $169.99
price for USA in USD
Softcover $139.99
price for USA in USD
About this book

This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Bruton’s tyrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia.  Patients’ management in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies are  discussed. The book’s closing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells.

Table of contents (7 chapters)

Table of contents (7 chapters)

Buy this book

eBook $109.00
price for USA in USD
  • ISBN 978-3-319-22714-6
  • Digitally watermarked, DRM-free
  • Included format: EPUB, PDF
  • ebooks can be used on all reading devices
  • Immediate eBook download after purchase
Hardcover $169.99
price for USA in USD
Softcover $139.99
price for USA in USD
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Bibliographic Information

Bibliographic Information
Book Title
Agammaglobulinemia
Editors
  • Alessandro Plebani
  • Vassilios Lougaris
Series Title
Rare Diseases of the Immune System
Series Volume
4
Copyright
2015
Publisher
Springer International Publishing
Copyright Holder
Springer International Publishing Switzerland
eBook ISBN
978-3-319-22714-6
DOI
10.1007/978-3-319-22714-6
Hardcover ISBN
978-3-319-22713-9
Softcover ISBN
978-3-319-37249-5
Series ISSN
2282-6505
Edition Number
1
Number of Pages
IX, 119
Topics