Hillard M. Lazarus Alvin H. Schmaier Editors Concise Guide to Hematology Second Edition 123 Concise Guide to Hematology Hillard M. Lazarus • Alvin H. Schmaier Editors Concise Guide to Hematology Second Edition Editors Hillard M. Lazarus Alvin H. Schmaier Department of Medicine Department of Medicine Case Western Reserve University University Hospital Cleveland Medical Center Cleveland, OH Cleveland, OH USA USA ISBN 978-3-319-97872-7 ISBN 978-3-319-97873-4 (eBook) https://doi.org/10.1007/978-3-319-97873-4 Library of Congress Control Number: 2018959739 © 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 Dr. Lazarus dedicates this textbook to his loving wife (Joan) of more than 40 years, to their sons and spouses (Jeffrey and Jana; Adam and Sarah), and to their grandchildren (Jacob, Jillian, Aaron, and Benny). Dr. Schmaier dedicates this book to Linda and to the next generation of physicians in his family Alec, Lauren, Eleanor, and Stella. Preface The third edition of our textbook will provide easy-access and up-to-date information in the complex, diverse, and rapidly changing field of hematology and hematologic malignancy. We target entry-level postdoctoral fellows in hematology who are initiating their training as well as medical students and medical interns and residents, clinical nurse practitioners, and physi- cian assistants working in the field of hematology-oncology. We also believe our text will be of considerable use to medical technologists, pharmacy personnel, and pharmaceutical repre- sentatives and medical science liaisons. Finally, this book will be attractive to persons working in blood banking and transfusion medicine as well as personnel in cardiology, pathology, infectious disease, and general pediatrics. The text is presented in an easy-to-read, textual outline format to facilitate rapid and clear information in an organized fashion. This approach differs from other products and allows the reader to locate sections of immediate interest without wading through entire paragraphs to obtain the desired data. Further, the electronic version can be accessed with an “APP.” For optimal effect, we utilize a robust indexing tool. Each chapter is written by a recognized expert(s). The diverse range of authors adds rich- ness on the extent of the progress made in the respective disciplines. The contributors discuss the disorder/disease entity, beginning from incidence/prevalence, to include pathophysiology and patient demographics. Patient-, disease-, and treatment-related issues are discussed. The reader is provided with up-to-date information but also is given insight and the tools to be able to continue the study of these topics as they develop over time. It is our intent to make this volume the first choice for information for the neophyte who enters the field of hematology. Hillard M. Lazarus Department of Medicine Case Western Reserve University, Cleveland, OH, USA Alvin H. Schmaier Department of Medicine Case Western Reserve University, Cleveland, OH, USA Department of Medicine University Hospitals Cleveland Medical Center Cleveland, OH, USA vii Contents 1 Introduction to Hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Alvin H. Schmaier 2 Hematopoiesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Gabriel Ghiaur and Richard J. Jones 3 Red Blood Cell Biochemistry and Physiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Eduard J. van Beers and Richard van Wijk 4 Anemia: Clinical Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Peter W. Marks 5 Iron Deficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 Gordon D. McLaren 6 Vitamin B (Cobalamin) and Folate Deficiency . . . . . . . . . . . . . . . . . . . . . . . . . . 37 12 Aśok C. Antony 7 Sickle Cell Disease and the Hemoglobinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . 49 Lydia H. Pecker, Carolyn Hoppe, and Jane A. Little 8 Congenital Hemolytic Anemias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 Xylina Gregg, Archana Agarwal, and Josef T. Prchal 9 Acquired Hemolytic Anemias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 Theodosia A. Kalfa 10 Physiology of Hemostasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81 Alvin H. Schmaier 11 Approach to the Bleeding Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Alvin H. Schmaier 12 Congenital Bleeding Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95 Rohith Jesudas and Steven W. Pipe 13 Acquired Bleeding Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111 Marcel Levi 14 Platelet Function in Hemostasis and Inherited Disorders of Platelet Number and Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 A. Koneti Rao and Natthapol Songdej 15 Acquired Thrombocytopenia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 Theodore E. Warkentin, Siraj Mithoowani, and Donald M. Arnold 16 Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Sargam Kapoor, Mukesh K. Jain, and Lalitha Nayak ix x Contents 17 Anticoagulants and Treatment of Venous Thromboembolism . . . . . . . . . . . . . . . 163 Allison Burnett and Jack Ansell 18 Antiplatelet Therapy in Cardiovascular Disease . . . . . . . . . . . . . . . . . . . . . . . . . . 195 Alec A. Schmaier and Deepak L. Bhatt 19 Physiological Roles of Leukocytes and Disorders . . . . . . . . . . . . . . . . . . . . . . . . . 209 Jahnavi Gollamudi, Alex Y. Huang, and Evi X. Stavrou 20 Pediatric Hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 225 Sanjay P. Ahuja 21 Bone Marrow Structure and Marrow Aspiration, Biopsy, and Collection for Therapeutic Intent Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233 Howard Meyerson, Suchitra Sundaram, and Hillard M. Lazarus 22 Flow Cytometry in Hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Howard Meyerson 23 Genetics in Hematologic Disorders: Implications of Recurring Chromosome Abnormalities and Gene Mutations . . . . . . . . . . . . . . . . . . . . . . . . 277 Yanming Zhang 24 Understanding Molecular Testing in Patients Affected by Hematologic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299 Christopher Ryder, Menglei Zhu, and Navid Sadri 25 Chronic Myeloid Leukemia (CML) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Thomas G. Knight, Michael R. Grunwald, and Edward A. Copelan 26 Myeloproliferative Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323 Emma J. Gudgin and Anna L. Godfrey 27 Myelodysplastic Syndromes (MDS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333 Yazan F. Madanat and Mikkael A. Sekeres 28 Acute Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343 Kamal Menghrajani and Martin S. Tallman 29 Classification of Lymphomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355 Colin McHugh, Kerry Schaffer, and Carla Casulo 30 Clinical Evaluation and Management of Hodgkin Lymphoma . . . . . . . . . . . . . . 371 Nmazuo W. Ozuah and Ann S. LaCasce 31 Indolent Lymphomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379 Thomas M. Habermann 32 Aggressive Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387 Timothy Fenske and Jonathan T. Kapke 33 Clinical Evaluation and Management of Chronic Lymphocytic Leukemia . . . . 401 Nitin Jain and Susan O’Brien 34 Plasma Cell Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Taimur Sher and Morie A. Gertz Contents xi 35 Neoplastic Hematologic Disorders in Children and Adolescents . . . . . . . . . . . . . 423 Rupert Handgretinger and Michaela Döring 36 Transfusion Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463 Emily K. Storch, Brian S. Custer, Jay E. Menitove, and Paul D. Mintz 37 Hematopoietic Cell Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487 Gordon L. Phillips II and Hillard M. Lazarus 38 Infections in Hematology Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503 Ola Blennow and Per Ljungman Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 519 Contributors Archana Agarwal Department of Pathology, University of Utah Health, Salt Lake City, UT, USA Sanjay P. Ahuja Pediatric Hematology/Oncology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, OH, USA Jack Ansell Hofstra Northwell School of Medicine, Hempstead, NY, USA Aśok C. Antony Division of Hematology-Oncology, Department of Medicine, Indiana University School of Medicine, Indiana University Hospitals and Roudebush Veterans Affairs Medical Center, Indianapolis, IN, USA Donald M. Arnold Department of Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, ON, Canada Deepak  L.  Bhatt Brigham and Women’s Hospital, Heart & Vascular Center, Boston, MA, USA Ola  Blennow Department of Infectious Diseases, Karolinska University Hospital, Stockholm, Sweden Allison  Burnett University of New Mexico Hospital Inpatient Pharmacy Department, Albuquerque, NM, USA Carla Casulo James P. Wilmot Cancer Institute, University of Rochester, Rochester, NY, USA Edward A. Copelan Levine Cancer Institute, Atrium Health, Charlotte, NC, USA Brian S. Custer UCSF Department of Laboratory Medicine, Blood Systems Research Institute, San Francisco, CA, USA Michaela  Döring Department of General Pediatrics, Hematology/Oncology, University Children‘s Hospital Tübingen, Tübingen, Germany Timothy Fenske Division of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI, USA Morie A. Gertz Mayo Clinic, Jacksonville, FL, USA Gabriel Ghiaur The Sidney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA Anna  L.  Godfrey Department of Haematology and Haematopathology and Oncology Diagnostics Service, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK Jahnavi Gollamudi Department of Medicine, Hematology and Oncology Division, University Hospitals Cleveland Medical Center, Cleveland, OH, USA xiii