Titolo: Paroxysmal Nocturnal Hemoglobinuria

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Paroxysmal Nocturnal Hemoglobinuria

From Bench to Bedside

Editore

Springer

Anno

2017

Pagine

358

ISBN

9784431560012

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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.

A History of Research of PNH: Defining a Disease

Rosse, Wendell Franklyn

Pages 1-8
Synthesis, Genetics, and Congenital Diseases of GPI-Anchored Proteins

Maeda, Yusuke (et al.)

Pages 11-54
Animal Models of Paroxysmal Nocturnal Hemoglobinuria

Murakami, Yoshiko (et al.)

Pages 55-65
Complement and PNH

Parker, Charles J.

Pages 67-95
Epidemiology in PNH: The PNH Global Registry

Brodsky, Robert A.

Pages 99-107

Epidemiology in PNH: Clinical Epidemiological Studies in Korea

Lee, Jong Wook

Pages 109-120
Haemolysis in PNH: Depletion of Nitric Oxide

Hill, Anita

Pages 121-135
Bone Marrow Failure in PNH

Höchsmann, Britta (et al.)

Pages 137-151
Thrombophilia in PNH

Ninomiya, Haruhiko (et al.)

Pages 153-172
Diagnosis and Classification of PNH

Nakakuma, Hideki (et al.)

Pages 173-183
Clinical Significance of a Small Population of Glycosylphosphatidylinositol-Anchored Membrane Proteins (GPI-APs)-Deficient Cells in the Management of Bone Marrow Failure

Nakao, Shinji

Pages 185-195
Clonal Origin and Clonal Selection in PNH

Luzzatto, Lucio

Pages 197-213
Pathogenesis of Clonal Dominance in PNH: Selection Mechanisms in PNH

Kawaguchi, Tatsuya (et al.)

Pages 215-227
Pathogenesis of Clonal Dominance in PNH: Growth Advantage in PNH

Inoue, Norimitsu (et al.)

Pages 229-251
Clinical Management in PNH

Shichishima, Tsutomu (et al.)

Pages 253-269
Clinical Effects of Eculizumab in PNH

Szer, Jeffrey

Pages 271-282
Clinical Effects of Eculizumab in PNH: Extravascular Hemolysis After Eculizumab Treatment

Notaro, Rosario (et al.)

Pages 283-295
Clinical Effects of Eculizumab in PNH: Poor Responders to Eculizumab

Nishimura, Jun-ichi (et al.)

Pages 297-306
Hematopoietic Stem Cell Transplantation in PNH

Socié, Gérard (et al.)

Pages 307-317
Future Strategies of Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria

Risitano, Antonio M.

Pages 319-346
Pregnancy in Paroxysmal Nocturnal Hemoglobinuria

Miyasaka, Naoyuki (et al.)

Pages 347-358