Humoral Primary Immunodeficiencies
This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is
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Mario Milco D'Elios · Marta Rizzi Editors
Humoral Primary Immunodeficiencies
Rare Diseases of the Immune System Series Editors: Lorenzo Emmi Domenico Prisco Editorial Board: Systemic Vasculitis L. Emmi C. Salvarani R. A. Sinico
Primary Immunodeficiency A. Plebani C. T. Baldari M. M. D’Elios
Autoimmune Disease P. L. Meroni D. Roccatello M. Matucci Cerinic L. Emmi
Systemic Fibroinflammatory Disorders A. Vaglio
Autoinflammatory Syndromes M. Gattorno F. De Benedetti R. Cimaz
Sentiero al mattino Laura Maddii Emmi (Private collection)
Mario Milco D’Elios • Marta Rizzi Editors
Humoral Primary Immunodeficiencies
Editors Mario Milco D’Elios Careggi Hospital Department of Clinical and Experimental Medicine, University of Florence Firenze Italy
Marta Rizzi University Medical Centre Freiburg Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency Freiburg Germany
ISSN 2282-6505 ISSN 2283-6403 (electronic) Rare Diseases of the Immune System ISBN 978-3-319-91784-9 ISBN 978-3-319-91785-6 (eBook) https://doi.org/10.1007/978-3-319-91785-6 Library of Congress Control Number: 2018962559 © Springer Nature Switzerland AG 2019 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. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Dedicated with love to our children Emilia, Carlo, and Flavia and Sofia, Emanuele, Alessandro, and Bernardo; to all of our patients with primary immunodeficiencies and their families; to our nurses; and to all of our students.
Preface
Primary immunodeficiencies are rare diseases. Nevertheless, the study of patients with primary immunodeficiencies has provided the chance to understand how the immune system really works. This journey into the pathogenetic mechanisms behind primary imm
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