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Paroxysmal Nocturnal Hemoglobinuria

From Bench to Bedside

  • Book
  • © 2017

Overview

Editors:
  1. Yuzuru Kanakura

    1. Department of Hematology and Ocology, Osaka University Graduate School of Medicine, Suita, Japan

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  2. Taroh Kinoshita

    1. Immunology Frontier Research Center, Osaka University, Suita, Japan

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  3. Jun-ichi Nishimura

    1. Dept of Hematology and Ocology, Osaka University Dept of Hematology and Ocology, Suita, Japan

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  • Reviews advances in research and treatment of paroxysmal nocturnal hemoglobinuria (PNH)

  • Examines the role of eculizumab in management strategies

  • Overviews briefly the history and basic and clinical science of PNH

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Table of contents (21 chapters)

  1. Front Matter

    Pages i-viii
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  2. A History of Research of PNH: Defining a Disease

    • Wendell Franklyn Rosse
    Pages 1-8

  3. Basic Sciences in PNH

    1. Front Matter

      Pages 9-9
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    2. Synthesis, Genetics, and Congenital Diseases of GPI-Anchored Proteins

      • Yusuke Maeda, Yoshiko Murakami, Taroh Kinoshita
      Pages 11-54

    3. Animal Models of Paroxysmal Nocturnal Hemoglobinuria

      • Yoshiko Murakami, Taroh Kinoshita
      Pages 55-65

    4. Complement and PNH

      • Charles J. Parker
      Pages 67-95

  4. Clinical Sciences in PNH

    1. Front Matter

      Pages 97-97
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    2. Epidemiology in PNH: The PNH Global Registry

      • Robert A. Brodsky
      Pages 99-107

    3. Epidemiology in PNH: Clinical Epidemiological Studies in Korea

      • Jong Wook Lee
      Pages 109-120

    4. Haemolysis in PNH: Depletion of Nitric Oxide

      • Anita Hill
      Pages 121-135

    5. Bone Marrow Failure in PNH

      • Britta Höchsmann, Hubert Schrezenmeier
      Pages 137-151

    6. Thrombophilia in PNH

      • Haruhiko Ninomiya, Anita Hill
      Pages 153-172

    7. Diagnosis and Classification of PNH

      • Hideki Nakakuma, Tsutomu Shichishima, Jun-ichi Nishimura
      Pages 173-183

    8. Clinical Significance of a Small Population of Glycosylphosphatidylinositol-Anchored Membrane Proteins (GPI-APs)-Deficient Cells in the Management of Bone Marrow Failure

      • Shinji Nakao
      Pages 185-195

    9. Clonal Origin and Clonal Selection in PNH

      • Lucio Luzzatto
      Pages 197-213

    10. Pathogenesis of Clonal Dominance in PNH: Selection Mechanisms in PNH

      • Tatsuya Kawaguchi, Hideki Nakakuma
      Pages 215-227

    11. Pathogenesis of Clonal Dominance in PNH: Growth Advantage in PNH

      • Norimitsu Inoue, Taroh Kinoshita
      Pages 229-251

    12. Clinical Management in PNH

      • Tsutomu Shichishima, Hideyoshi Noji
      Pages 253-269

    13. Clinical Effects of Eculizumab in PNH

      • Jeffrey Szer
      Pages 271-282

    14. Clinical Effects of Eculizumab in PNH: Extravascular Hemolysis After Eculizumab Treatment

      • Rosario Notaro, Antonio M. Risitano
      Pages 283-295
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Keywords

About this book

This volume reviews the fundamental understanding of this potentially life-threatening disease and the advances in treatment that have been achieved with the use of the monoclonal antibody eculizumab. Although the PIGA gene has been known for many years, the mechanism of clonal dominance in paroxysmal nocturnal hemoglobinuria is still largely unknown. This book, Paroxysmal Nocturnal Hemoglobinuria, discusses the direction of continuing research in this area, as well as the potential for the development of management guidelines. It serves as a valuable source of information for both basic scientists and physicians, especially immunologists targeting GPI-anchored proteins and complements, and hematologists specializing in bone marrow failure.

Editors and Affiliations

  • Department of Hematology and Ocology, Osaka University Graduate School of Medicine, Suita, Japan

    Yuzuru Kanakura

  • Immunology Frontier Research Center, Osaka University, Suita, Japan

    Taroh Kinoshita

  • Dept of Hematology and Ocology, Osaka University Dept of Hematology and Ocology, Suita, Japan

    Jun-ichi Nishimura

About the editors

Editors:

Yuzuru Kanakura, Osaka University, Suita, Japan


Taroh Kinoshita, Osaka University, Suita, Japan



Jun-ichi Nishimura, Osaka University, Suita, Japan

Bibliographic Information

  • Book Title: Paroxysmal Nocturnal Hemoglobinuria

  • Book Subtitle: From Bench to Bedside

  • Editors: Yuzuru Kanakura, Taroh Kinoshita, Jun-ichi Nishimura

  • DOI: https://doi.org/10.1007/978-4-431-56003-6

  • Publisher: Springer Tokyo

  • eBook Packages: Medicine, Medicine (R0)

  • Copyright Information: Springer Japan KK 2017

  • Hardcover ISBN: 978-4-431-56001-2Published: 24 January 2017

  • Softcover ISBN: 978-4-431-56751-6Published: 15 July 2018

  • eBook ISBN: 978-4-431-56003-6Published: 16 January 2017

  • Edition Number: 1

  • Number of Pages: VIII, 358

  • Number of Illustrations: 25 b/w illustrations, 46 illustrations in colour

  • Topics: Hematology, Immunology

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