Paroxysmal Nocturnal Hemoglobinuria From Bench to Bedside
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,
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Paroxysmal Nocturnal Hemoglobinuria
Yuzuru Kanakura • Taroh Kinoshita Jun-ichi Nishimura Editors
Paroxysmal Nocturnal Hemoglobinuria From Bench to Bedside
Editors Yuzuru Kanakura Department of Hematology and Oncology Osaka University Graduate School of Medicine Suita, Osaka, Japan Jun-ichi Nishimura Department of Hematology and Oncology Osaka University Graduate School of Medicine Suita, Osaka, Japan
Taroh Kinoshita Research Institute for Microbial Diseases Osaka University Suita, Osaka, Japan WPI Immunology Frontier Research Center Osaka University Suita, Osaka, Japan
ISBN 978-4-431-56001-2 ISBN 978-4-431-56003-6 (eBook) DOI 10.1007/978-4-431-56003-6 Library of Congress Control Number: 2016961209 © Springer Japan KK 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer Japan KK The registered company address is: Chiyoda First Bldg. East, 3-8-1 Nishi-Kanda, Chiyoda-ku, Tokyo 101-0065, Japan
Preface
On this occasion we hereby publish the only book in the world that focuses on paroxysmal nocturnal hemoglobinuria (PNH). PNH arises as a consequence of clonal expansion of hematopoietic stem cells that have acquired a somatic mutation in the phosphatidylinositol glycan class A (PIGA) gene. The resulting hematopoietic cells are deficient in glycosylphosphatidylinositol (GPI)-anchored proteins, including the complement regulatory proteins CD55 and CD59; this accounts for the intravascular hemolysis that is the primary clinical manifestation of PNH. The PIGA gene was identified by our group at Osaka University in 1993, and understanding of the pathophysiology of PNH has made remarkable progress since then. To this day, we Japanese researchers have made a significant contribution and have led the world in PNH research, together with colleagues worldwide. PNH is an ultra-rare disease with a frequency of a few people in 100,000, and for a long time ther
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