Primary Hematology

473 Pages·2001·31.627 MB·English

by  Ayalew Tefferi MD (auth.), Ayalew Tefferi MD (eds.)

PRIMARY HEMATOLOGY
PRIMARY HEMATOLOGY
Edited by
AYALEW TEFFERI, MD
Consultant, Division o[H ematology and Internal Medicine, Mayo Clinic
and Mayo Foundation; Associate Professor o[M edicine, Mayo MedicalSchool;
Rochester, Minnesota
Springer Science+Business Media, LLC
ISBN 978-1-61737-122-6 ISBN 978-1-59259-228-9 (eBook)
DOI 10.1007/978-1-59259-228-9
Copyright © 2001 Springer Science+Business Media New York.
Originally published by Mayo Foundation for Medical Education and Research in 2001.
Softcover reprint ofthe hardcover 1s t edition 2001
For additional copies, pricing for bulk purchases, and/or information about other Humana titles, contact Humana at
999 Riverview Drive, Suite 208, Totowa, NJ 07512 or at any ofthe following numbers: Tel: [phone]; Fax:
973-256-8341; E-mail: [email protected] or visit our website at http://humanapress.com
All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval
system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise,
without written permission from Mayo Foundation.
As new scientific information becomes available through basic and clinical research, recommended treatments and drug
therapies undergo changes. The authors and publisher have made all reasonable attempts to make this book accurate,
up to date, and in accord with accepted standards at the time of publication. The authors, editor, and publisher are not
responsible for errors or omissions or for consequences from application ofthe book, and make no warranty, expressed
or implied, in regard to the contents ofthe book. Any practice described in this book should be applied by the reader
in consultation with a physician and taking regard ofthe unique circumstances that may apply in each situation. The
reader is advised always to check product information (package inserts) for changes and new information regarding
dose and contraindications before administering any drug. Caution is especially urged when using new or infrequently
ordered drugs. Nothing in this publication implies that Mayo Clinic endorses the products or equipment mentioned in
this book.
All artic\es, comments, opinions, conclusions, or recommendations are those ofthe author(s) and do not necessarily reflect
the views ofthe publisher or of Mayo Clinic.
Cover design by Patricia F. Cleary.
Cover illustration by Garry Post.
This publication is printed on acid-free paper. ®
ANSI Z39.48-1984 (American National Standards Institute)
Permanence of Paper for Printed Library Materials.
Photocopy Authorization PoIicy:
Authorization tophotocopy items for internal orpersonal use, orthe internat orpersonal use ofspecific clients, is granted
by Humana Press Inc., provided that the base fee of US $10.00 per copy, plus US $00.25 per page, is paid directly to
the Copyright Clearance Center at 222 Rosewood Drive, Danvers, MA 01923. For those organizations that have been
granted a photocopy Iicense from the CCC, a separate system ofpayment has been arranged and is acceptable to Humana
Press Inc. The fee code for users ofthe Transactional Reporting Service is: [0-89603-664-2/01 $10.00 + $00.25].
10 9 8 7 6 5 4 3 2 I
Library of Congress Cataloging-in-Publication Data
PREFACE
The discipline ofhematology is enriched by tremendous diversity in pathology and its
close identification with molecular biology. Routine clinical practice often involves the
diagnosis and management of anemia, sickle cell disorders, hypercoagulable states, and
monoclonal gamrnopathy ofundetermined significance. Hematologic malignancies, in
cluding lymphoma and leukemia, are some ofthe most treatable cancers, and treatment
outcome has been improved by the use of hematopoietic stern cell transplantation. He
matologists, oncologists, cardiologists, intemists, and family practitioners are all equally
interested in the problems ofthrombosis and bleeding. Transfusion medicine, the use of
hematopoietic growth factors, and cancer chemotherapy have all become integral com
ponents ofhematology practice. A holistic approach to patient care requires so me back
ground information on ethics and statistics. This book was written with all of these
considerations in mind.
I thank the authors ofthe chapters for their diligence in providing fine contributions.
I am appreciative of the efforts of the Section of Scientific Publications at Mayo Clinic
in the production ofthis book, especially Virginia A. Dunt, Mary L. Schwager, Mary K.
Horsman, Roberta J. Schwartz, and LeAnn M. Stee.
Ayalew Tefferi, M.D.
v
To
Dr. Rohert L. Phyliky
and
Dr. H. Clark Hoagland
decent human beings, master clinicians, and loyal friends
LIST OF COLOR PLATES
Color plates appear in an insert foIlowing p. 242.
Platel: Chapter 3, Fig. IB. Hemoglobin moleeule.
Plate 2: Chapter 3, Fig. lC. Globin genes.
Plate 3: Chapter 3, Fig. 2A Peripheral smear-ß-thalassemia minor.
Plate 4: Chapter 3, Fig. 2B. Peripheral smear-ß-thalessemia major.
Plate 5: Chapter 3, Fig. 2C. Peripheral smear-hemoglobin H disease.
Plate 6: Chapter 4, Fig. 2A. Peripheral smear-sickle ceIl disease.
Plate 7: Chapter 7, Fig. 1. Ecchymoses and petechiae from severe thrombocy
topenia.
Plate 8: Chapter 7, Fig. 2. Platelet rosetting in the presence of EDT A.
Plate 9: Chapter 10, Fig. 2. Facial plethora in a patient with polycythemia vera.
Plate 10: Chapter 10, Fig. 3. Retinal vein distention in a patient with poly
cythemia vera, before and after phlebotomy.
Plates 11, 12: Chapter 10, Fig. 4A,B. Massive splenomegaly in a patient with
myelofibrosis.
Plate 13: Chapter 11, Fig. 1. Dimorphie red ceIls.
Plate 14: Chapter 11, Fig. 2. Pseudo Pelger-Huet granulocyte.
Plate 15: Chapter 11, Fig. 3. Dyserythopoiesis seen in bone marrow aspirate.
Plate 16: Chapter 14, Fig. 1. Heterogeneity in the hematopoietic stern ceIl origin
of acute myelogenous leukemia (AML).
Plate 17: Chapter 15, Fig. 2. Blood and marrow transplantation (BMT) process.
Plate 18: Chapter 15, Fig. 4A. The human leukocyte antigen gene system on the
short arm of chromosome 6 consists of the class I region of A and B loci as weIl as
other loci and the class 11 region of D loci. The class 11 region contains genes of the
complement system.
Plate 19: Chapter 15, Fig. 6A. Chronic graft-versus-host disease with lichenoid
and sclerodermatous changes in the face and scalp with alopecia.
Plate 20: Chapter 15, Fig. 6B. Lichenoid hyperpigmentation of the back.
Vll
CONTENTS
Preface ............................................................................................................................ v
List of Color Plates ..................................................................................................... vii
Contributors ................................................................................................................ xiii
I 1
ANEMIA AND OTHER CYTOPENIAS ......................................
A Practical Approach to the Diagnosis of Anemia .......................... 3
Ayalew Tefferi, MD
2 Specific Anemia Syndromes and Their Management.. .................. 21
Ayalew Tefferi, MD
3 The Thalassemia Syndromes and the Hemoglobinopathies .......... 41
Angela Dispenzieri, MD
4 Sickle Cell Disorders ...................................................................... 65
Angela Dispenzieri, MD
5 Practical Aspects in the Diagnosis and Management
of Aplastic Anemia ..................................................................... 83
RafaelFonseca, MD
6 Differential Diagnosis ofNeutropenia ........................................... 93
Costas L. Constantinou, MD
7 Causes and Management of Isolated Thrombocytopenia ............ 105
Gerardo Colon-Otero, MD
11
PRIMARY AND SECONDARY MYELOPROLIFERATIVE
DISORDERS .... ............ ..................... ............. 117
8 An Approach to the Patient With Erythrocytosis ......................... 119
Aya/ew Tefferi, MD
9 Differential Diagnosis and Management ofThrombocytosis ...... 129
Lawrence A. So/berg, Jr., MD, PhD
10 Chronic Myeloproliferative Diseases:
Polycythemia Vera and Agnogenic Myeloid Metaplasia ........ 137
Ayalew Tefferi, MD
11 An Approach to the Patient With Myelodysplasia ....................... 149
Pierre Noel, MD
12 The Eosinophil and Eosinophilia .................................................. 161
Joseph H. Butterfieid, MD
ix
x Contents
III
LEUKEMIAS AND HEMATOPOIETIC STEM CELL
173
TRANSPLANTATION .............................. .........
13 Leukocytosis and the Chronic Leukemias ................................... 175
Ayalew TeJJeri, MD
14 Acute Leukemia ............................................................................ 193
Mark R. Litzow, MD
15 Blood and Marrow Transplantation .............................................. 209
Mark R. Litzow, MD
IV 229
LYMPHOID AND PlASMA CELL DISORDERS ••••••••••••••••••••
16 Lymphadenopathy ......................................................................... 231
Thomas M. Habermann, MD
17 Hodgkin' s Disease and N on-Hodgkin' s Lymphoma ................... 241
Thomas M. Habermann, MD
18 An Evaluation of Patients With Monoclonal Gammopathies ...... 255
Robert A. Kyle, MD
19 Multiple Myeloma and Related Disorders ................................... 269
Robert A. Kyle, MD
V 289
THROMBOTIC AND BLEEDING DISORDERS ••••••••••••••••••••••
20 An Overview of the Hemostatic System and Interpretation
of Common Screening Coagulation Tests ............................... 291
Rajiv K. Pruthi, MD
21 Bleeding Disorders: An Overview and Clinical Practice ........... 303
Rajiv K. Pruthi, MD
22 Thrombogenesis and Thrombotic Disorders ................................ 317
Rajiv K. Pruthi, MD
VI 337
MISCELLANEOUS TOPICS IN HEMATOLOGY ••••••••••••••••••••
23 Transfusion Medicine: Practical Issues ....................................... 339
S. Breanndan Moore, MD
24 Hereditary Hemochromatosis: A Disease for All Physicians ..... 351
Virgil F. Fairbanks, MD
25 Hematologic Manifestations of HIV Disease .............................. 365
Zelalem Ternesgen, MD
26 Hematopoietic Growth Factors in Clinical Practice .................... 371
Pierre Noe/, MD
27 A Primer of Chemotherapy ........................................................... 379
Alex A. Adjei, MD, PhD
Contents Xl
28 An Approach to Porphyria ............................................................ 407
Lawrence A. So/berg, Jr., MD, PhD
29 Ethical Issues in the Care of Patients With Hematologic
Diseases .................................................................................... 415
C. Christopher Hook, M.D.
30 Statistical Methods Frequently Used in Hematology
Research .................................................................................... 431
Georgene Schroeder, MS
Index ........................................................................................................................... 451