Table Of ContentWilliams Hematology
Malignant
Lymphoid
Diseases
Oliver W. Press, MD, PhD John P. Leonard, MD
Giuliani/Press Endowed Chair in Cancer Research Richard T. Silver Distinguished Professor of Hematology and
Fred Hutchinson Cancer Research Center Medical Oncology
Professor of Medicine and Bioengineering Joan and Sanford I. Weill Department of Medicine
University of Washington Weill Cornell Medical College
Seattle, Washington New York Presbyterian Hospital
New York, New York
Marshall A. Lichtman, MD
Professor Emeritus of Medicine
(Hematology-Oncology) and of Biochemistry
and Biophysics
Dean, Emeritus School of Medicine and Dentistry
University of Rochester Medical Center
Rochester, New York
New York Chicago San Francisco Athens London Madrid Mexico City
Milan New Delhi Singapore Sydney Toronto
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William J. Williams, MD
1926 – 2016
Medical educator, investigator, physician, mentor, academic leader,
colleague, and the founding editor of Williams Hematology
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3 4
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1 . Transmission electron micrograph (TEM) of a normal blood lymphocyte. 2. Scanning electron micrograph (SEM) of a normal blood lymphocyte.
3. TEM of Sézary cell in a patient with the erythrodermic type of cutaneous T-cell lymphoma. Note the cell’s characteristic profoundly misshaped
(cerebriform) nucleus. 4. TEM of a hairy cell. Arrow indicates a ribosome-lamella complex. This structure is not specifc for hairy cell leukemia but is
found in a variable proportion of hairy cells in about 50 percent of cases examined by TEM. Frequent cytoplasmic membrane, “hairy,” projections.
5. TEM of plasmablast (undiferentiated myeloma cell). Arrow points to a Russell body. 6. A lymphoblast from the marrow of a patient with acute
lymphoblastic leukemia. Very high nuclear-to-cytoplasmic ratio. Prominent nucleolus. The nucleus is virtually all euchromatin (likely transcriptionally active).
(Reproduced with permission from Lichtman’s Atlas of Hematology, www.accessmedicine.com.)
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v
CONTENTS
Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii 12. Marginal Zone B-Cell Lymphomas . . . . . . . . . . . . . . . . . . . . . . . 175
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .xi Pier Luigi Zinzani and Alessandro Broccoli
13. Burkitt Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
1. Classifcation of Malignant Lymphoid Disorders . . . . . . . . . . . . . 1 Carla Casulo, Jonathan W. Friedberg, and Andrew G. Evans
Robert A. Baiocchi
14. C utaneous T-Cell Lymphoma (Mycosis Fungoides and
2. Acute Lymphoblastic Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Sézary Syndrome). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191
Richard A. Larson Larisa J. Geskin and Christina C. Patrone
3. Chronic Lymphocytic Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . 37 15. Mature T-Cell and Natural Killer Cell Lymphomas . . . . . . . . . 207
Farrukh T. Awan and John C. Byrd Neha Mehta-Shah, Alison Moskowitz, and Steven Horwitz
4. Hairy Cell Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 16. Plasma Cell Neoplasms: General Considerations . . . . . . . . . . . 223
Michael R. Grever and Gerard Lozanski G uido Tricot, Siegfried Janz, Kalyan Nadiminti, Erik Wendlandt,
5. Large Granular Lymphocytic Leukemia . . . . . . . . . . . . . . . . . . . . 73 and Fenghuang Zhan
Pierluigi Porcu and Aharon G. Freud 17. Essential Monoclonal Gammopathy . . . . . . . . . . . . . . . . . . . . . . 237
6. General Considerations of Lymphomas: Epidemiology, Marshall A. Lichtman
Etiology, Heterogeneity, and Primary Extranodal Disease. . . . . 81 18. Myeloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249
Oliver W. Press and Marshall A. Lichtman E lizabeth O’Donnell, Francesca Cottini, Noopur Raje,
7. Pathology of Lymphomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 and Kenneth Anderson
Randy D. Gascoyne and Brian F. Skinnider 19. Immunoglobulin Light-Chain Amyloidosis . . . . . . . . . . . . . . . . 291
8. Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 MFroarnicei sA K. G. Beurtazd, iTaimur Sher, Angela Dispenzieri, and
Oliver W. Press and John P. Leonard
20. Macroglobulinemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
9. Difuse Large B-Cell Lymphoma and Related Neoplasms . . . . 137 S teven P. Treon, Jorge J. Castillo, Zachary R. Hunter, and
Stephen D. Smith and Oliver W. Press Giampaolo Merlini
10. Follicular Lymphoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 21. Heavy-Chain Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
Oliver W. Press and John P. Leonard Dietlind L. Wahner-Roedler and Robert A. Kyle
11. Mantle Cell Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165 I ndex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 31
Martin Dreyling
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vii
CONTRIBUTORS
Kenneth Anderson, MD Angela Dispenzieri, MD
Dana-Farber Cancer Institute Division of Hematology
Boston, Massachusetts Mayo Clinic
Rochester, Minnesota
Farrukh T. Awan, MD
Associate Professor of Internal Medicine Martin Dreyling, MD
Division of Hematology Department of Internal Medicine III
Department of Internal Medicine Medical Center of the University of Munich
Te Ohio State University Comprehensive Cancer Center Munich, Germany
Columbus, Ohio
Andrew G. Evans, MD, PhD
Robert A. Baiocchi, MD, PhD Assistant Professor
Associate Professor of Medicine Department of Pathology and Laboratory Medicine
Division of Hematology University of Rochester Medical Center
Department of Internal Medicine Rochester, New York
Te Ohio State University
Columbus, Ohio Aharon G. Freud, MD, PhD
Assistant Professor
Alessandro Broccoli, MD Department of Pathology
Institute of Hematology “L. e A. Seràgnoli” Te Ohio State University
University of Bologna Columbus, Ohio
Bologna, Italy
Jonathan W. Friedberg, MD
Francis K. Buadi, MD Samuel Durand Professor of Medicine
Division of Hematology Director, Wilmot Cancer Institute
Mayo Clinic University of Rochester Medical Center
Rochester, Minnesota Rochester, New York
John C. Byrd, MD Randy D. Gascoyne, MD, FRCPC
D. Warren Brown Chair of Leukemia Research Clinical Professor of Pathology
Professor of Medicine, Medicinal Chemistry, and Veterinary Research Director, Centre for Lymphoid Cancers
Biosciences Departments of Pathology and Advanced Terapeutics
Director, Division of Hematology British Columbia Cancer Agency, the BC Cancer Research Center, and
Department of Medicine University of British Columbia
Te Ohio State University Vancouver, British Columbia, Canada
Columbus, Ohio
Morie A. Gertz, MD, MACP
Jorge J. Castillo, MD Division of Hematology
Assistant Professor of Medicine Mayo Clinic
Dana-Farber Cancer Institute Rochester, Minnesota
Harvard Medical School
Boston, Massachusetts Larisa J. Geskin, MD, FAAD
Associate Professor of Dermatology and Medicine
Carla Casulo, MD Director, Division of Cutaneous Oncology and Comprehensive Skin
Assistant Professor of Medicine Cancer Center
James P. Wilmot Cancer Institute Department of Dermatology
University of Rochester Medical Center Columbia University
Rochester, New York New York, New York
Francesca Cottini, MD
Dana-Farber Cancer Institute
Boston, Massachusetts
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viii Contributors Contributors ix
Michael G. Grever, MD Neha Mehta, MD
Chair and Professor Department of Medicine
Department of Internal Medicine Memorial Sloan Kettering Cancer Center
Bertha Bouroncle MD and Andrew Pereny Chair in Medicine New York, New York
Te Ohio State University
Columbus, Ohio Giampaolo Merlini, MD
Director, Center for Research and Treatment of Systematic
Steven Horwitz, MD Amyloidosis
Department of Medicine University Hospital Policlinico San Meteo
Memorial Sloan Kettering Cancer Center Professor, Department of Medicine
New York, New York University of Pavia
Pavia, Italy
Zachary R. Hunter, PhD
Bing Center for Waldenstrom’s Macroglobulinemia Alison Moskowitz, MD
Dana-Farber Cancer Institute Department of Medicine
Instructor of Medicine, Harvard Medical School Memorial Sloan Kettering Cancer Center
Boston, Massachusetts New York, New York
Siegfried Janz, MD, DSc Kalyan Nadiminti, MD
Division of Hematology/Oncology and Blood and Division of Hematology/Oncology and Blood and
Marrow Transplantation Marrow Transplantation
Department of Pathology, Carver College of Medicine Department of Pathology, Carver College of Medicine
University of Iowa Health Care University of Iowa Health Care
Iowa City, Iowa Iowa City, Iowa
Robert A. Kyle, MD Elizabeth O’Donnell, MD
Professor of Medicine Massachusetts General Hospital
Laboratory Medicine and Pathology Boston, Massachusetts
Mayo Clinic
Rochester, Minnesota Christina C. Patrone, BA
Columbia University College of Physicians and Surgeons
Richard A. Larson, MD New York, New York
Section of Hematology/Oncology
Department of Medicine and the Comprehensive Cancer Center Pierluigi Porcu, MD
University of Chicago Professor of Internal Medicine
Chicago, Illinois Division of Hematology and Comprehensive Cancer Center
Te Ohio State University
John P. Leonard, MD Columbus, Ohio
Richard T. Silver Distinguished Professor of Hematology and
Medical Oncology Oliver W. Press, MD, PhD
Joan and Sanford I. Weill Department of Medicine Giuliani/Press Endowed Chair in Cancer Research
Weill Cornell Medical College Fred Hutchinson Cancer Research Center
New York Presbyterian Hospital Professor of Medicine and Bioengineering
New York, New York University of Washington
Seattle, Washington
Marshall A. Lichtman, MD
Professor Emeritus of Medicine and of Biochemistry and Biophysics Noopur Raje, MD
Dean Emeritus, School of Medicine and Dentistry Massachusetts General Hospital
University of Rochester Medical Center Boston, Massachusetts
Rochester, New York
Taimur Sher, MD
Gerard Lozanski, MD Division of Hematology/Oncology
Director, Hematopathology Mayo Clinic
Medical Director Jacksonville, Florida
Flow Cytometry Laboratory
Associate Professor—Clinical
Department of Pathology
Te Ohio State University
Columbus, Ohio
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viii Contributors Contributors ix
Brian F. Skinnider, MD Dietlind L. Wahner-Roedler, MD
Clinical Associate Professor Professor of Medicine
Department of Pathology Mayo Clinic
Vancouver General Hospital, British Columbia Cancer Agency, and Rochester, Minnesota
University of British Columbia
Vancouver, British Columbia, Canada Erik Wendlandt, PhD
Division of Hematology/Oncology and Blood and
Stephen D. Smith, MD Marrow Transplantation
Seattle Cancer Care Alliance Department of Pathology, Carver College of Medicine
Seattle, Washington University of Iowa Health Care
Iowa City, Iowa
Steven P. Treon
Director, Bing Center for Waldenstrom’s Macroglobulinemia Fenghuang Zhan, MD, PhD
Dana-Farber Cancer Institute Division of Hematology/Oncology and Blood and
Associate Professor, Harvard Medical School Marrow Transplantation
Boston, Massachusetts Department of Pathology, Carver College of Medicine
University of Iowa Health Care
Guido Tricot, MD, PhD Iowa City, Iowa
Division of Hematology/Oncology and Blood and
Marrow Transplantation Pier Luigi Zinzani, MD, PhD
Department of Pathology, Carver College of Medicine Professor
University of Iowa Health Care Institute of Hematology “L. e A. Seràgnoli”
Iowa City, Iowa University of Bologna
Bologna, Italy
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