Immune Hematology Diagnosis and Management of Autoimmune Cytopenias
This text provides a concise yet comprehensive overview of the most common autoimmune cytopenias affecting adults and children. The book is divided into four sections, each of which focuses on a major autoimmune cytopenia. The first section features backg
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Immune Hematology
Jenny M. Despotovic Editor
Immune Hematology Diagnosis and Management of Autoimmune Cytopenias
Editor Jenny M. Despotovic Baylor College of Medicine Texas Children's Hospital Houston, TX USA
ISBN 978-3-319-73268-8 ISBN 978-3-319-73269-5 (eBook) https://doi.org/10.1007/978-3-319-73269-5 Library of Congress Control Number: 2018939554 © Springer International Publishing AG, part of Springer Nature 2018 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. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Printed on acid-free paper This Springer imprint is published by the registered company Springer International Publishing AG part of Springer Nature. The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Preface
The autoimmune cytopenias are a fascinating and clinically challenging group of disorders characterized by the development of autoantibodies against self-antigens on the surface of hematopoietic cells. The pathophysiology of these disorders is complex and incompletely understood but is generally related to loss of self- tolerance due to immune dysregulation. These disorders can be primary or secondary to underlying systemic autoimmunity or immune deficiency. The diagnosis of these disorders can be challenging and varies significantly according to disease and underlying pathology. While antibody testing is a cornerstone of diagnosis in some autoimmune cytopenias, it offers low sensitivity and specificity for others. This book offers an extensive overview of immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), Evans syndrome, thrombotic thrombocytopenic purpura (TTP), and autoimmune neutropenia (AIN). Each section contains chapters covering historical background, pathophysiology, differential diagnosis, diagnostic workup, and management, offering a much more in-depth review of these disorders than is available in most hematology or immunol
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