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Paroxysmal Nocturnal Hemoglobinuria and Related Disorders

Molecular Aspects of Pathogenesis

  • Conference proceedings
  • © 2003

Overview

Editors:
  1. Mitsuhiro Omine

    1. Division of Hematology, Internal Medicine Showa University Fujigaoka Hospital, Aoba-ku, Yokohama, Japan

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  2. Taroh Kinoshita

    1. Department of Immunoregulation, Research Institute for Microbial Diseases Osaka University, Suita, Osaka, Japan

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    You can also search for this editor in PubMed   Google Scholar

  • Provides an invaluable summary of current research on the fundamental aspects of PNH pathology, presented by renowned experts in the field

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Table of contents (32 papers)

  1. Front Matter

    Pages II-XVIII
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  3. Pathogenesis and Animal Models of PNH

    1. Front Matter

      Pages 25-25
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    2. Mice with Blood Cells Deficient in GPI-linked Proteins — The Generation of a Murine Model to Investigate the Pathogenesis and Pathophysiology of Paroxysmal Nocturnal Hemoglobinuria (PNH)

      • Monica Bessler, Shashikant Kulkarni, Marek Jasinski, Anne Schaefer, Peter Keller, Gabi Tremml
      Pages 27-39

    3. A Multistep Model for the Pathogenesis and Evolution of PNH

      • Russell E. Ware, Matthew M. Heeney, Chrisley V. Pickens, Carlos M. DeCastro, Thad A. Howard
      Pages 41-53

    4. Proposals for Classification of the Clinical Stages, Grading of Severity and the Molecular Pathogenesis of Paroxysmal Nocturnal Hemoglobinuria

      • Tsutomu Shichishima
      Pages 55-68

  4. Clinical Pathology and Natural History of PNH

    1. Front Matter

      Pages 69-69
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    2. A Short History of Paroxysmal Nocturnal Hemoglobinuria: How We Came To Understand Its Natural History

      • Wendell F. Rosse
      Pages 71-83

    3. Clinical Pathology and natural history of PNH; The French Society of Hematology experience.

      • GĂ©rard SociĂ©, Jean-Yves Mary
      Pages 85-94

  5. Mechanism of Clonal Expansion in PNH

    1. Front Matter

      Pages 95-95
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    2. PNH clone acquires both a survival and a growth advantage?

      • Hideki Nakakuma, Tatsuya Kawaguchi
      Pages 97-103

    3. The Role of Lymphoid Cells in the Pathogenesis of PNH

      • Lucio Luzzatto, Anastasios Karadimitris, David Araten, Rosario Notaro
      Pages 105-116

    4. A Possible Intrinsic Mechanism for Clonal Expansion of PNH Abnormal Cells

      • Norimitsu Inoue, Tomohisa Izui, Maki Kuwayama, Jun-ichi Nishimura, Ken Kurokawa, Takashi Machii et al.
      Pages 117-126

  6. PNH Clones Under Bone Marrow Failure

    1. Front Matter

      Pages 127-127
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    2. Clinical Significance of Increased PNH-Type Cells in the Peripheral Blood of Patients with Aplastic Anemia and Refractory Anemia

      • Shinji Nakao, Hong-bo Wang, Tatsuya Chuhjo
      Pages 129-137

    3. Incidence and Clinical Significance of PNH clone in Myelodysplastic Syndromes

      • Masao Tomonaga, Masako Iwanaga, Kengo Fuchigami, Yoriko Inoue, Tatsuro Joh, Itsuro Jinnai
      Pages 139-148

    4. The Enigma of the Aplastic Anemia/PNH Syndrome

      • Neal S. Young, Jaroslaw P. Maciejewski, Elaine Sloand, Guiben Chen, Weihua Zeng, Antonio Risitano et al.
      Pages 149-158

  7. PNH, Aplastic Anemia, Myelodysplastic Syndromes (1): Pathology

    1. Front Matter

      Pages 159-159
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Keywords

About this book

Few publications focus on the mysterious, genetically acquired disease paroxysmal nocturnal hemoglobinuria (PNH) and the related "intractable" disorders—aplastic anemia and myelodysplastic syndromes. Now, however, the latest understanding of the clinical and molecular genetic aspects of PNH is summarized here in the proceedings of the International Symposium held in Tokyo in 2001. Major topics reviewed include the molecular mechanisms of the PIG-A gene mutation; complement activation and inhibitors; experimental animal models; pathogenesis; the history of PNH research; the natural history of the disease; the mechanism of PNH clone expansion; the emergence of PNH clones under bone marrow failure syndromes; and treatment of the disease by immunosuppressive agents and stem cell transplantation. This book provides an invaluable summary of current research on the fundamental aspects of PNH pathology, presented by renowned experts in the field.

 

Editors and Affiliations

  • Division of Hematology, Internal Medicine Showa University Fujigaoka Hospital, Aoba-ku, Yokohama, Japan

    Mitsuhiro Omine

  • Department of Immunoregulation, Research Institute for Microbial Diseases Osaka University, Suita, Osaka, Japan

    Taroh Kinoshita

Bibliographic Information

  • Book Title: Paroxysmal Nocturnal Hemoglobinuria and Related Disorders

  • Book Subtitle: Molecular Aspects of Pathogenesis

  • Editors: Mitsuhiro Omine, Taroh Kinoshita

  • DOI: https://doi.org/10.1007/978-4-431-67867-0

  • Publisher: Springer Tokyo

  • eBook Packages: Springer Book Archive

  • Copyright Information: Springer Japan 2003

  • Hardcover ISBN: 978-4-431-70329-7Published: 09 December 2002

  • Softcover ISBN: 978-4-431-68004-8Published: 12 October 2012

  • eBook ISBN: 978-4-431-67867-0Published: 06 December 2012

  • Edition Number: 1

  • Number of Pages: XVIII, 285

  • Topics: Hematology

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