Table Of ContentHemostasis and Thrombosis
Hemostasis and
Thrombosis
Practical Guidelines in
Clinical Management
EDITED BY
Hussain I. Saba MD, PhD
Professor of Medicine
Director of Hematology/Hemophilia/Hemostasis & Thrombosis Center
USF College of Medicine;
Professor Emeritus
Department of Malignant Hematology
Moffitt Cancer Center and Research Institute
Tampa, FL, USA
Harold R. Roberts MD
Emeritus Professor of Medicine and Pathology
Division of Hematology/Oncology
University of North Carolina
Chapel Hill, NC, USA
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Library of Congress Cataloging-in-Publication Data
Hemostasis and thrombosis (Saba)
Hemostasis and thrombosis : practical guidelines in clinical management / edited by Hussain I.
Saba, Harold R. Roberts.
p. ; cm.
Includes bibliographical references and index.
ISBN 978-0-470-67050-7 (pbk.)
I. Saba, Hussain I., editor of compilation. II. Roberts, H. R. (Harold Ross), editor of
compilation. III. Title.
[DNLM: 1. Blood Coagulation Disorders–therapy. 2. Hemostatic Disorders–
therapy. 3. Thrombosis–therapy. WH 322]
RC647.C55
616.1'57–dc23
2013042714
A catalogue record for this book is available from the British Library.
Wiley also publishes its books in a variety of electronic formats. Some content that appears in
print may not be available in electronic books.
Cover image: iStock photo. File #16045826 © Eraxion
Cover design by Meaden Creative
Set in 9.5/13 pt MeridienLTStd Roman by Toppan Best-set Premedia Limited
1 2014
Contents
Contributors, vii
Preface, x
Acknowledgments, xi
1 Theories of Blood Coagulation: Basic Concepts and Recent
Updates, 1
Dougald M. Monroe and Maureane Hoffman
2 Vascular Endothelium, Influence on Hemostasis: Past and
Present, 14
Hussain I. Saba and Sabiha R. Saba
3 Coagulation Testing: Basic and Advanced Clinical Laboratory
Tests, 30
Nils Egberg and Margareta Blombäck
4 Factor VIII Deficiency or Hemophilia A: Clinical Bleeding and
Management, 45
Pier Mannuccio Mannucci and Massimo Franchini
5 Factor IX Deficiency or Hemophilia B: Clinical Manifestations and
Management, 57
Raj S. Kasthuri, Harold R. Roberts, and Paul E. Monahan
6 Factor XI Deficiency or Hemophilia C, 71
Charles E. Bane, Anne T. Neff, and David Gailani
7 Factor VIII and IX Inhibitors in Hemophilia, 82
Meera Chitlur and Jeanne Lusher
8 Treatment Options for Acquired Hemophilia, 93
Anjali Sharathkumar and David Green
9 Factor XII Deficiency or Hageman Factor Deficiency, 112
Evi X. Stavrou and Alvin H. Schmaier
10 Inherited Combined Factor Deficiency States, 127
Asma Latif and Louis Aledort
v
vi Contents
11 Acute and Chronic Immune Thrombocytopenia: Biology, Diagnosis,
and Management, 137
Samir Dalia and Benjamin Djulbegovic
12 Disseminated Intravascular Coagulation: Diagnosis and
Management, 151
Stephanie J. Davis and Craig M. Kessler
13 Mechanisms of Fibrinolysis and Basic Principles of Management, 169
John W. Weisel and Rustem I. Litvinov
14 Post-thrombotic Syndrome, 186
Jean-Philippe Galanaud and Susan R. Kahn
15 Von Willebrand Disease: Clinical Aspects and Practical
Management, 197
Francesco Rodeghiero, Alberto Tosetto, and Giancarlo Castaman
16 Platelets in Hemostasis: Inherited and Acquired Qualitative
Disorders, 209
Noman Ashraf and Hussain I. Saba
17 Contributions of Platelet Polyphosphate to Hemostasis and
Thrombosis, 236
James H. Morrissey
18 Thrombotic Microangiopathy: Biology, Diagnosis, and
Management, 246
Samir Dalia and Hussain I. Saba
19 Hemostasis and Aging, 259
Lodovico Balducci
20 Hemostatic Problems in Chronic and Acute Liver Disease, 271
Ton Lisman and Robert J. Porte
21 Cancer and Thrombosis, 284
Erica A. Peterson and Agnes Y. Y. Lee
22 An Update on Low-Molecular-Weight Heparins, 296
Jawed Fareed, Debra Hoppensteadt, and Walter P. Jeske
Index, 315
Color plate section can be found facing page 212
Contributors
Louis Aledort MD, MACP Meera Chitlur MD
The Mary Weinfeld Professor of Clinical Associate Professor of Pediatrics and Director of
Research in Hemophilia Hemophilia/Hemostasis Clinic
Division of Hematology and Medical Oncology Wayne State University
The Tisch Cancer Institute Children’s Hospital of Michigan
Mount Sinai School of Medicine Detroit, MI, USA
New York, NY, USA
Samir Dalia MD
Noman Ashraf MD Fellow in Hematology and Oncology
Assistant Professor H. Lee Moffitt Cancer & Research Center
Department of Hematology/Oncology University of South Florida
University of South Florida/James A. Haley VA Tampa, FL, USA
Hospital
Tampa, FL, USA Stephanie J. Davis, MD
Resident
Lodovico Balducci MD Department of Internal Medicine
Professor of Oncologic Sciences University of North Carolina Hospitals
H. Lee Moffit Cancer Center & Research Chapel Hill, NC, USA
Institute
Tampa, FL, USA Benjamin Djulbegovic MD, PhD
Distinguished Professor
Charles E. Bane Jr. DVM University of South Florida & H. Lee Moffitt
Department of Pathology, Microbiology, and Cancer Center & Research Institute
Immunology Tampa, FL, USA
Vanderbilt University
Nashville, TN, USA Nils Egberg MD, PhD
Associate Professor
Margareta Blombäck MD, PhD Department of Molecular Medicine and
Professor Emeritus Surgery
Department of Molecular Medicine and Division of Clinical Chemistry and Blood
Surgery Coagulation
Division of Clinical Chemistry and Blood Karolinska Institutet
Coagulation Stockholm, Sweden
Karolinska Institutet
Stockholm, Sweden Jawed Fareed PhD
Professor of Pathology & Pharmacology
Giancarlo Castaman MD Hemostasis & Thrombosis Research
Consultant Hematologist Laboratories
Department of Cell Therapy and Hematology Loyola University Chicago
Hemophilia and Thrombosis Center Maywood, IL, USA
San Bortolo Hospital
Vicenza, Italy
vii
viii Contributors
Massimo Franchini, MD Craig M. Kessler MD, MACP
Director Professor of Medicine and Pathology
Department of Transfusion Medicine and Director, Division of Coagulation
Hematology Division of Hematology and Oncology
C. Poma Hospital Lombardi Comprehensive Cancer Center
Mantova, Italy Georgetown University Medical Center
Washington, DC, USA
David Gailani MD
Professor of Pathology, Microbiology and Asma Latif MD
Immunology Clinical Fellow
Division of Hematology/Oncology Division of Hematology and Medical Oncology
Vanderbilt University The Tisch Cancer Institute
Nashville, TN, USA Mount Sinai School of Medicine
New York, NY, USA
Jean-Philippe Galanaud MD
Assistant Professor of Vascular Medicine Agnes Y. Y. Lee MD, MSc, FRCPC
Clinical Investigation Centre and Department Medical Director, Thrombosis Program
of Internal Medicine Associate Professor of Medicine
Montpellier University Hospital Division of Hematology
Montpellier, France University of British Columbia
British Columbia Cancer Agency
David Green MD, PhD Vancouver, BC, Canada
Professor Emeritus of Medicine
Feinberg School of Medicine Ton Lisman PhD
Northwestern University Associate Professor
Chicago, IL, USA Section of Hepatobiliary Surgery and
Liver Transplantation
Maureane Hoffman MD, PhD Department of Surgery
Professor of Pathology University Medical Center Groningen
Duke University Medical Center University of Groningen
Durham, NC, USA Groningen, The Netherlands
Debra Hoppensteadt PhD Rustem I. Litvinov MD, PhD, DrSci
Professor of Pathology and Pharmacology Senior Research Investigator
Loyola University Medical Center Department of Cell and Developmental Biology
Maywood, IL, USA University of Pennsylvania
Perelman School of Medicine
Walter P. Jeske PhD Philadelphia, PA, USA
Professor of Thoracic and Cadiovascular
Surgery Jeanne M. Lusher MD
Loyola University Medical Center Distinguished Professor of Pediatrics and
Maywood, IL, USA Marion I Barnhart Chair in Hemostasis
Research
Susan R. Kahn MD, MSc, FRCPC Wayne State University
Professor of Medicine Children’s Hospital of Michigan
Division of Internal Medicine Detroit, MI, USA
Lady Davis Institute, Jewish General Hospital
Department of Medicine Pier Mannuccio Mannucci MD
McGill University Scientific Director
Montreal, QC, Canada IRCCS Cà Granda Foundation
Maggiore Policlinico Hospital
Raj S. Kasthuri MD Milan, Italy
Associate Professor
Department of Medicine
Division of Hematology/Oncology
University of North Carolina at Chapel Hill
Chapel Hill, NC, USA
