Blood and Bone Marrow Pathology: Expert Consult: Online and Print, 2nd Edition

690 Pages·2011·124.57 MB·English

by  Anna Porwit

Blood and
Bone Marrow

PATHOLOGY
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SECOND EDITION
Blood and
Bone Marrow
PATHOLOGY
Anna Porwit MD, PhD
Professor
Department of Pathology
Karolinska University Hospital and Institute
Stockholm, Sweden;
Department of Laboratory Medicine and Pathobiology
University of Toronto
Toronto, ON, Canada
Jeffrey McCullough MD
Professor of Laboratory Medicine and Pathology
American Red Cross Professor of Transfusion Medicine
University of Minnesota
Minneapolis, MN, USA
Wendy N. Erber MD, DPhil, FRCPA, FRCPath
Consultant Hematologist
Addenbrooke’s Hospital;
Hematology Department
University of Cambridge
Cambridge, UK
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First edition 2002
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Churchill Livingstone
British Library Cataloguing in Publication Data
Blood and bone marrow pathology.
1. Blood – Diseases. 2. Bone marrow – Diseases. 3. Blood – Diseases – Histopathology. 4. Bone marrow –
Diseases – Histopathology. I. Porwit, Anna. II. McCullough, Jeffrey J. III. Erber, Wendy N., 1957–
616.1′5 – dc22
ISBN-13: 9780702031472
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Preface
In the time since the first edition of Blood and Bone Marrow major driving force behind Blood and Bone Marrow Pathology.
Pathology was published in 2002, major advances have been It is with immense sadness we note the untimely death of
made in all facets of the field of hematology. Much of this Sunitha during the production of this second edition. His
has resulted from progress in molecular genetics which has death has been a tremendous loss to hematology.
enhanced our understanding of the pathological basis of The book is divided into sections on normal blood and
hematological disorders. The knowledge of the molecular bone marrow, pathology of the bone marrow, disorders of
basis of hematological diseases is now being used to both erythroid cells and leukocytes, abnormalities of hemostasis
diagnose and classify non-malignant and malignant hema- and immunohematology. Within each section there are
tological disorders. This has had a major impact on the chapters devoted to specific aspects of each of these areas
hemopoietic tumors where the World Health Organization and which have been written by internationally recognized
classification is in large part based on this new biological experts. We are most grateful to them for their outstanding
information. This second edition of Blood and Bone Marrow contributions and the time they have devoted to this project.
Pathology has therefore been significantly updated and We are also immensely grateful to the publishers and espe-
revised to incorporate these advances. Many chapters have cially Michael Houston, Ben Davie and Rachael Harrison for
been totally rewritten to encapsulate the advances in the their enormous help in bringing this project to fruition. We
understanding of the fundamentals of the pathology of hope this second edition of Blood and Bone Marrow Pathology
blood and bone marrow in health and disease. This book will be of benefit to clinical and laboratory hematologists,
truly focuses on the pathology of blood and bone marrow. hematopathologists and those in training in gaining a better
Although clinical aspects of disease are alluded to along with understanding of the pathology of blood and bone marrow.
treatment approaches, this is only given as a guide to the
reader. The book is aimed at bridging pure cytology and Anna Porwit
histology with clinical hematology diagnostics. Jeffrey McCullough
The book was originally to be co-edited by Professor Wendy N. Erber
Sunitha Wickramasinghe, co-editor of the first edition and a 2011
Contributors
Kenneth C. Anderson MD, PhD Scott D. Boyd MD, PhD
Director, Jerome Lipper Multiple Myeloma Center Assistant Professor of Pathology
Dana-Farber Cancer Institute Department of Pathology
Kraft Family Professor of Medicine Stanford University
Harvard Medical School Stanford, CA, USA
Boston, MA, USA
Shannon M. Buckley PhD
Daniel A. Arber MD Postdoctoral Research Fellow
Professor and Associate Chair of Pathology Department of Pathology
Department of Pathology New York University Medical Center
Stanford University New York, NY USA
Stanford, CA, USA
Guntram Büsche
Donald M. Arnold MDCM, MSc, FRCPC Lecturer in Hematopathology
Assistant Professor, Department of Medicine Institute of Pathology
Division of Hematology and Thromboembolism Medizinische Hochschule Hannover
Michael G. DeGroote School of Medicine Hannover, Germany
McMaster University
Magdalena Czader MD, PhD
Canadian Blood Services
Director, Division of Hematopathology
Hamilton, ON, Canada
Department of Pathology and Laboratory Medicine
Barbara J. Bain MB BS, FRACP, FRCPath Indiana University School of Medicine
Professor in Diagnostic Hematology Indianapolis, IN, USA
Imperial College
Geoff Daniels PhD, FRCPath
Honorary Consultant Hematologist
Head of Molecular Diagnostics and Senior Research Fellow
Department of Hematology
Bristol Institute for Transfusion Sciences and IBGRL
St Mary’s Hospital
NHS Blood and Transplant
London, UK
Bristol, UK
Marie Christine Béné Pharm Sci D, PhD
Faith E. Davies MBBCh, MD, MRCP, FRCPath
Professor of Immunology
Hemato-oncology Unit
Immunology Laboratory
Royal Marsden Hospital
Faculty of Medicine
London, UK
Nancy University and University Hospital
Vandoeuvre-lès-Nancy, France Jean Delaunay MD, PhD
Professor of Genetics
David H. Bevan FRCP FRCPath
INSERM
Director and Consultant Hematologist
Faculté de Médecine Paris-Sud
Centre for Hemostasis and Thrombosis
Univ Paris-Sud
Guy’s and St Thomas’ NHS Foundation Trust
Le Kremlin-Bicêtre, France
London, UK
Wendy N. Erber MD, DPhil, FRCPA, FRCPath
Oliver Bock MD
Consultant Hematologist
Professor for Transplantation Pathology
Addenbrooke’s Hospital
Head Central Tissue and Specimen Bank
Hematology Department
Institute of Pathology
University of Cambridge
Medizinische Hochschule Hannover
Cambridge, UK
Hannover, Germany
Gines Escolar MD, PhD
Jeremiah C. Boles MD
Head of Department
Hematology/Oncology Fellow
Servicio de Hemoterapia y Hemostasia
Division of Hematology/Oncology
Hospital Clinic, University of Barcelona, Medical School
University of North Carolina at Chapel Hill
Barcelona, Spain
Chapel Hill, NC, USA
David J. P. Ferguson BSc, PhD, DSc
Gerben Bouma PhD
Professor of Ultrastructural Morphology
Research Associate
Nuffield Department of Clinical Laboratory Science
Molecular Immunology Unit
University of Oxford
UCL Institute of Child Health
John Radcliffe Hospital
London, UK
Oxford, UK
Contributors
Edward C. Gordon-Smith MA, BSc, FRCP, FRCPath, FMedSci D. Mark Layton FRCP, FRCPCH
Emeritus Professor of Hematology Consultant and Reader in Hematology
St George’s College, University of London Department of Hematology
London, UK Imperial College
London, UK
Ralph Green MD, PhD, FRCPath
Professor and Chair Emeritus Peter K. MacCallum MD, FRCP, FRCPath
Department of Pathology and Laboratory Medicine Department of Hematology
University of California Davis School of Medicine Wolfson Institute of Preventive Medicine
Sacramento, CA, USA Barts and The London School of Medicine and Dentistry
Queen Mary College, University of London
Carolyn S. Grove MBBS, FRACP, FRCPA
London, UK
Clinical Research Training Fellow
Wellcome Trust Sanger Institute Alison May PhD
Hinxton, UK Senior Research Fellow
Department of Hematology
Georgina W. Hall MBBS, PhD, FRCP, FRCPath, FRCPCH
Cardiff University School of Medicine
Consultant Paediatric Hematologist
Cardiff, UK
Honorary Senior Lecturer
Paediatric Hematology/Oncology Unit Jeffrey McCullough MD
Children’s Hospital Professor of Laboratory Medicine and Pathology
John Radcliffe Hospital American Red Cross Professor of Transfusion Medicine
Oxford, UK University of Minnesota
Minneapolis, MN, USA
Nancy M. Heddle MSc., FCSMLS(D)
Professor Mufaddal T. Moonim MD, FRCPath
Michael G. DeGroote School of Medicine Consultant Histopathologist
Department of Medicine, Department of Pathology and Department of Histopathology
Molecular Medicine Guy’s and St Thomas’ Hospitals
McMaster University London, UK
Hamilton, ON, Canada
Jane C. Moore BSc, ART
Hans-Peter Horny MD Assistant Professor
Professor of Pathology Michael G. DeGroote School of Medicine
Institute of Pathology Department of Medicine, Department of Pathology and
Ansbach, Germany Molecular Medicine
McMaster University
Carolyn Katovich Hurley PhD, D(ABHI)
Hamilton, ON, Canada
Professor of Oncology and Microbiology and Immunology
Georgetown University Medical Center Ishac Nazi PhD
Washington, DC, USA Assistant Professor
Michael G. DeGroote School of Medicine
John G. Kelton MD, FRCPC
Department of Medicine, Department of Pathology and
Professor
Molecular Medicine
Department of Medicine
Department of Biochemistry and Biomedical Sciences
Department of Pathology and Molecular Medicine
McMaster University
Michael G. DeGroote School of Medicine
Hamilton, ON, Canada
McMaster University
Hamilton, ON, Canada Adrian C. Newland CBE, MA, FRCP, FRCPath
Professor of Hematology
Nigel S. Key MB, ChB, FRCP
Department of Hematology
Harold R Roberts Distinguished Professor
The Royal London Hospital
Department of Medicine, Division of Hematology/Oncology
London, UK
University of North Carolina at Chapel Hill
Chapel Hill, NC, USA Eva Norström MD, PhD
Medical Doctor
Hans H. Kreipe MD
Department of Laboratory Medicine
Professor of Pathology, Director Institute of Pathology
Clinical Chemistry Malmö
Medizinische Hochschule Hannover
Lund University
Hannover, Germany
Lund, Sweden
Hans M. Kvasnicka MD
Attilio Orazi MD, FRCPath
Professor of Pathology
Professor of Pathology and Laboratory Medicine and Vice-
Senkenberg Institute of Pathology
Chair for Hematopathology
University of Frankfurt
Director, Division of Hematopathology
Frankfurt, Germany
Department of Pathology and Laboratory Medicine
Weill Medical College of Cornell University
New York, NY, USA
x
Contributors
David J. Perry MD PhD, FRCPEdin, FRCPLond,FRCPath Swee Lay Thein MB, BS, FRCP, FRCPath, DSc, FMedSci
Consultant Hematologist Professor of Molecular Hematology/Consultant Hematologist
Department of Hematology King’s College London/King’s College Hospital NHS
Addenbrooke’s Hospital Foundation Trust
Cambridge, UK James Black Centre
London, UK
Martin J. Pippard BSc, MB, ChB, FRCP, FRCPath
Emeritus Professor of Hematology Adrian J. Thrasher PhD, FRCP, FRCPath, FMedSci
University of Dundee Professor of Paediatric Immunology
Dundee, UK Molecular Immunology Unit
UCL Institute of Child Health
Anna Porwit MD, PhD
London, UK
Professor
Department of Pathology Jon D. van der Walt MB, BCh, FRCPat
Karolinska University Hospital and Institute Consultant Histopathologist
Stockholm, Sweden Department of Histopathology
Department of Laboratory Medicine and Pathobiology Guy’s and St Thomas’ Hospitals
University of Toronto London, UK
Toronto, ON, Canada
Catherine Verfaillie MD
Phillip E. Posch PhD Professor of Medicine
Assistant Professor of Oncology Director
Georgetown University School of Medicine Stamcelinstituut
Department of Oncology Katholieke Universiteit Leuven
Washington, DC, USA Leuven, Belgium
Drew Provan MD, FRCP, FRCPath James G. White MD
Academic Hematology Unit – Pathology Group Regents Professor
Blizard Institute of Cell and Molecular Science Departments of Laboratory Medicine and Pathology, and
Barts and the London School of Medicine and Dentistry Pediatrics
London, UK University of Minnesota School of Medicine
Minneapolis, MN, USA
Raffaele Renella MD, PhD
MRC Molecular Hematology Unit The late Sunitha N. Wickramasinghe
Weatherall Institute of Molecular Medicine Formerly Emeritus Professor of Hematology, University of
University of Oxford, UK London
Children’s Hospital Boston Visiting Professor of Hematology, University of Oxford
Harvard Medical School Formerly Head of the Department of Hematology
Boston, MA, USA St Mary’s Hospital Medical School
Imperial College of Science, Technology and Medicine
David R. Roper MSc, CSci, FIBMS
London, UK
Principal Biomedical Scientist
Department of Hematology Bridget S. Wilkins MB BCh, DM, PhD, FRCPath
Hammersmith Hospital Consultant Hematopathologist
Imperial College Healthcare NHS Trust Department of Cellular Pathology
London, UK Guy’s and St Thomas’ Hospitals NHS Foundation Trust
London, UK
Richard Rosenquist MD, PhD
Professor of Molecular Hematology William G. Wood PhD
Department of Genetics and Pathology Professor of Molecular Hematology
Rudbeck Laboratory MRC Molecular Hematology Unit
Uppsala University Weatherall Institute of Molecular Medicine
Uppsala, Sweden University of Oxford
John Radcliffe Hospital
Benny Sørensen MD, PhD
Oxford, UK
Director of HRU
Honorary Lecturer Gina Zini MD, PhD
Associate Professor Professor of Clinical Pathology
Hemostasis Research Unit (HRU) Institute of Hematology
Centre for Hemostasis and Thrombosis Catholic University of Sacred Heart
Guy’s and St Thomas’ NHS Foundation Trust Rome, Italy
St Thomas’ Hospital
London, UK
xi
Dedication
to Sunitha N. Wickramasinghe
human bone marrow, first published in 1973 and revised
in 1975, are still widely utilized by scientists and clinical
hematologists eager to understand the fundamentals of
this work. Sunitha was also a superb methodologist. In the
last 20 years, he became the leading authority on electron
microscopy of the blood forming tissues. Driven by his
scientific interest, he was always a hard worker. When he
visited me in Ulm to analyze the specimens of my collection
of bone marrow of patients with congenital dyserythropoi-
etic anemia, he started work early each morning; all members
of the electron microscopy department were highly impressed
by his immense skill, knowledge and enthusiasm in
this field.
I last met Sunitha in November 2008 at the second sym-
posium on congenital dyserythropoietic anemia. Although
already very ill and suffering from complications of recent
chemotherapy he gave two lectures. On the night after his
last presentation, he was admitted to the local hospital in a
small town on Lake Como in Italy. He returned to England
and with the support of his wife Priyanthi (to whom he had
dedicated his monograph) survived another six months.
Sunitha was a talented teacher, keen to share his knowl-
Sunitha N. Wickramasinghe, co-editor of the first edition of edge with students and younger colleagues. Not only were
this book, died in June 2009, after a long and brave fight his postgraduate courses in the United Kingdom legendary,
against a hematological neoplasm. but he also found the time to return to Sri Lanka for lectures
Sunitha was born in Sri Lanka (former Ceylon) in 1941, at the College of Hematologists. His intellectual curiosity
and received his medical training at the Royal College and and capacity for rigorous analysis, not only of the morphol-
Ceylon University. In 1964 he moved to England, and after ogy of blood cells but also of other clinical and laboratory
postgraduate clinical and research training in Cambridge observations, made him a highly respected partner of clini-
and Leeds was appointed as Professor of Hematology at St cians and their patients.
Mary’s Hospital in London, soon to become the Imperial Thanks to Sunitha’s friendly, always helpful, modest and
College Medical School. After retirement, he took a position charming personality he had many friends all over the world.
in the Weatherall Institute of Molecular Medicine at the We all miss him, but we are all happy to have had the oppor-
University of Oxford. He knew of his fatal disease in 2000, tunity to work with him and to know him and his family.
but continued to work on morphological and molecular
aspects of hematopoesis until 2008. Prof. Emerit. Dr. med.
Sunitha was an academic scholar in the genuine sense Hermann Heimpel FRCPath
of the word. This is evident in the more than 200 interna- Medizinische Universitätsklinik
tionally cited papers, mainly on abnormal red blood cell Ulm, Germany
for mation and the associated diseases. His monographs on 2011