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Biology and Management of Unusual Plasma Cell Dyscrasias Todd M. Zimmerman Shaji K. Kumar Editors Biology and Management of Unusual Plasma Cell Dyscrasias 123 Biology and Management of Unusual Plasma Cell Dyscrasias Todd M. Zimmerman • Shaji K. Kumar Editors Biology and Management of Unusual Plasma Cell Dyscrasias 123 Editors Todd M. Zimmerman, MD Shaji K. Kumar, MD Section of Hematology/Oncology Division of Hematology, The University of Chicago Department of Medicine Chicago, IL, USA Mayo Clinic Rochester, MN, USA ISBN 978-1-4419-6847-0 ISBN 978-1-4419-6848-7 (eBook) DOI 10.1007/978-1-4419-6848-7 Library of Congress Control Number: 2016940068 © Springer Science+Business Media New York 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 Science+Business Media LLC New York Preface The past decade has witnessed unprecedented advances in the understanding of monoclonal gammopathies, driven both by the availability of new therapeutic agents as well as a better under- standing of the biology of plasma cell disorders. While the focus in the field has been on multiple myeloma, its diagnosis, risk strati- fication, and therapies, we have also made significant progress in understanding the less common monoclonal gammopathies. Many of the lessons that we have learned in the context of multiple myeloma have widespread application for the less common plasma cell disorders. With testing for monoclonal proteins becoming more commonplace and the introduction of serum free light chain assay, allowing for detection of monoclonal proteins previously missed on traditional serum protein electrophoresis, monoclonal gammopathy and associated disorders are increasingly being diagnosed. While there are numerous publications in the area of multiple myeloma, very few review articles and book chapters are dedicated to the less common plasma cell disorders. This is a rapidly expanding area with more sensitive diagnostic technology, such as mass spectrometry, allowing us to identify small amounts of pro- tein hitherto not appreciated by the conventional diagnostic assays. Other advances in technology, such as next-generation sequencing as well as highly sensitive multiparameter flow cytometry, have v vi Preface contributed to our ability to detect and diagnose these rare plasma cell disorders. Not only has the ability to detect the small plasma cell clones been enhanced through these technologies, we have also become more aware of the various disease associations and the potential contribution of the monoclonal protein to disease mani- festations. This is particularly relevant in the context of many uncommon renal disorders that were not previously associated with monoclonal proteins. We have brought together many of the renowned experts in this field with the intent of developing a state-of-the-art reference that will allow clinicians and scientists to get a better understanding of these uncommon disorders. We have dedicated individual chapters to each of these disorders, with each chapters outlining what is currently known about the pathophysiology of these dis- orders as well as the common treatment approaches. We hope that the information in this book will not only help guide management of the patient in the clinic, but also form the basis for future studies related to these disorders. We sincerely hope that you will find this book to be a great addition to the literature in the field and a constant guide in your daily practice. Chicago, USA Todd M. Zimmerman, MD Rochester, USA Shaji K. Kumar, MD Contents 1 Plasma Cell Leukemia ......................... 1 Wilson I. Gonsalves and Shaji K. Kumar 2 Plasmacytoma—Current Approach to Diagnosis and Management ............................. 17 Nidhi Tandon and Shaji K. Kumar 3 POEMS Syndrome and Castleman’s Disease ....... 41 Angela Dispenzieri 4 Waldenstrom’s Macroglobulinemia............... 71 Stephen M. Ansell 5 Light Chain Amyloidosis ....................... 95 Amara S. Hussain and Anita D’Souza 6 Immunoglobulin Deposition Diseases ............. 109 Vinay Gupta, Wilson I. Gonsalves and Francis K. Buadi 7 Cryoglobulinemic Syndromes: Diagnosis and Management ............................. 127 Todd M. Zimmerman vii viii Contents 8 Idiopathic Systemic Capillary Leak Syndrome (Clarkson Disease) ............................ 143 Prashant Kapoor 9 Renal Disease Associated with Monoclonal Gammopathy ................................ 163 Nelson Leung and Samih H. Nasr Index .......................................... 195 Contributors Stephen M. Ansell, MD, PhD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Francis K. Buadi, MB, ChB Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Anita D’Souza, MD, MS Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, USA Angela Dispenzieri, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Wilson I. Gonsalves, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Vinay Gupta, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Amara S. Hussain, MD Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, USA Prashant Kapoor, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Shaji K. Kumar, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA ix x Contributors Nelson Leung, MD Departments of Nephrology and Hypertension, Hematology, Mayo Clinic, Rochester, MN, USA Samih H. Nasr, MD Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA Nidhi Tandon, MD Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Todd M. Zimmerman, MD Section of Hematology/Oncology, The University of Chicago, Chicago, IL, USA Abbreviations Ang 2 Angiopoietin 2 ASCT Autologous stem cell transplant C3 GN C3 Monoclonal associated glomerulonephritis HCDD Heavy chain deposition disease HMVECs Human microvascular endothelial cells ITG Immunotactoid glomerulonephropathy (ITG) IVIG Intravenous immunoglobulin LCDD Light chain deposition disease LHCDD Mixed light and heavy chain deposition disease MGUS Monoclonal gammopathy of undetermined significance PGNMID Proliferative glomerulonephritis with monoclonal IgG deposits SCLS Systemic capillary leak syndrome SNP Single nucleotide polymorphism VEGF Vascular endothelial growth factor xi Chapter 1 Plasma Cell Leukemia Wilson I. Gonsalves, MD and Shaji K. Kumar, MD Introduction Plasma cell dyscrasias account for roughly 10 % of all hematologic malignancies [1]. There is a wide spectrum of plasma cell neo- plasms that includes monoclonal gammopathy of undetermined significance (MGUS), smoldering myeloma (SMM), multiple myeloma (MM), and plasma cell leukemia (PCL) in the order of increasing malignant potential and aggressiveness. While circu- lating clonal plasma cells (PCs) can be seen in all stages including MGUS, they generally make up only a small fraction of the peripheral blood cells, with most of the clonal PCs are limited to the bone marrow. PCL on the other hand is characterized by the presence of a significant number of circulating clonal PCs. The first W.I. Gonsalves, MD (&) Á S.K. Kumar, MD Division of Hematology, Department of Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA e-mail: [email protected] S.K. Kumar, MD e-mail: [email protected] © Springer Science+Business Media New York 2017 1 T.M. Zimmerman and S.K. Kumar (eds.), Biology and Management of Unusual Plasma Cell Dyscrasias, DOI 10.1007/978-1-4419-6848-7_1 2 W.I. Gonsalves, MD and S.K. Kumar, MD case of PCL was described in 1906 by Gluzinski and Reichenstein [2]. However, it was not until 1974 that Kyle et al. defined it as the presence of more than 20 % PCs in the peripheral blood or an absolute PC count in the peripheral blood of greater than 2.0 × 109 cells/L [3, 4]. Primary PCL (pPCL) refers to those cases that originate de novo with no prior history of MM, whereas secondary PCL (sPCL) refers to cases where patients with a prior history of MM develop a leukemic phase of their MM. Epidemiology PCL is relatively rare and accounts for less than 5 % of all plasma cell neoplasms. In the United States, PCL has been estimated to have an incidence of 0.04–0.05/100,000 [5]. pPCL accounts for majority of the PCL cases (60–70 %) and sPCL being the remainder of cases [6]. sPCL occurs as a progressive
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