Consultative Hemostasis and Thrombosis

747 Pages·2007·16.889 MB·English

by  Craig S. Kitchens, MD, MACP, Barbara M. Alving, MD, MACP, and Craig M. Kessler, MD, MACP (Auth.)

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CONSULTATIVEHEMOSTASISANDTHROMBOSIS ISBN:978-1-4160-2401-9
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LibraryofCongressCataloging-in-PublicationData
Consultativehemostasisandthrombosis/[editedby]CraigS.Kitchens,BarbaraM.Alving,
CraigM.Kessler.—2nded.
p.;cm.
Includesbibliographicalreferencesandindex.
ISBN978-1-4160-2401-9
1. Bloodcoagulationdisorders. 2. Hemostasis. I. Kitchens,CraigS. II. Alving,BarbaraM.
III. Kessler,CraigM.
[DNLM: 1. BloodCoagulationDisorders. 2. Anticoagulants—therapeuticuse.
3. BloodPlateletDisorders. 4. FibrinolyticAgents—therapeuticuse. 5. Hemostasis—physiology.
6. Thrombosis. WH322C7582007]
RC647.C55C6552007 616.1057—dc22 2007007888
AcquisitionsEditor:DoloresMeloni
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PrintedintheUnitedStatesofAmerica
Lastdigitistheprintnumber: 9 8 7 6 5 4 3 2 1
DEDICATION
Dr. Harold R. Roberts
The editors dedicate the second edition of Consultative afellowshipintheDepartmentofPathologyatUNCunder
Hemostasis and Thrombosis to Dr. Harold R. Roberts, the direction of Dr. Kenneth Brinkhous. After completing
Professor of Pathology and Medicine at the University of his fellowship, Dr. Roberts became a research associate at
NorthCarolina(UNC)atChapelHill,inrecognitionofthe UNC;sevenyearslaterhewasnamedChiefoftheDivision
enduring contributions he has made to the diagnosis and of Hematology.
treatmentofpersonswithinheritedandacquireddisorders WhileexploringgeneticalterationsresultinginfactorIX
of hemostasis and thrombosis. His accomplishments are deficiency (hemophilia B), Dr. Roberts and his laboratory
theresultofalonganddistinguishedcareerasalaboratory investigators were the first to recognize that persons with
investigator,clinicalresearcher,andpracticingphysician. hemophiliaBcouldhaveanyoneofnumerouspointmuta-
A native of North Carolina, Dr. Roberts received both tionsordeletionsinthegenecodingforfactorIX.Thegene
his undergraduate training and his medical degree from alterationscouldresulteitherinnoexpressionofproteinor
UNC. His postgraduate training included an internship, in the expression of a protein that was recognized by
residency, and subspecialty training at the Vanderbilt immunologicmethodsbuthadlittleornofunctionalactivity.
School of Medicine in Nashville, Tennessee, and a year as Dr. Roberts’ laboratory also was the first to discover that
aFulbrightScholarinexperimentalpathologyinCopenha- a patient who presented with deficiencies of factors II,
gen, Denmark, where he worked in the area of fibrinolysis VII, IX, and X had a congenital deficiency of the enzymes
inthelaboratoryofProfessorTageAstrup.Hethenentered regulating the vitamin K–g-carboxylase system.
D As a clinical researcher, Dr Roberts focused on new and of Health, and he has been an invited guest lecturer
E
D improvedbiologicsforthetreatmentofbleedingdisorders. throughout North Carolina, the nation, and the world.
IC
A Histeamwasthefirsttotestcommerciallyprepared(large- Numerous societies and governments have honored
T
IO scale)plasmafractionsforthetreatmentoffactorVIIIdefi- Dr. Roberts for his laboratory and clinical research, as well
N
ciency (hemophilia A). These studies resulted in the devel- asfortheestablishmentofcentersforthetreatmentofdis-
opmentofpurifiedconcentratesoffactorVIII,markingthe orders of thrombosis or hemostasis. Dr. Roberts was the
beginningof anewerafor thetreatment ofhemophilia.In 2001 recipient of the Henry M. Stratton Medal presented
a natural evolution of this work, Dr. Roberts and his team by the American Society of Hematology. As a tribute to
conductedsomeofthefirstclinicaltrialswithrecombinant the outstanding care and advances in treatment that
factor VIII. His group also has been actively engaged in Dr. Roberts has provided for patients at the former UNC
gene therapy as a next step in improving treatment for Hemophilia Treatment Center, university officials have
patients with hemophilia. renamed the Center as the Harold R. Roberts Comprehen-
Dr. Roberts has played a seminal role in defining the sive Hemophilia Diagnostic and Treatment Center.
mechanism of action of recombinant factor VIIa (rFVIIa), TheoutstandingacademicworkofDr.Robertsisevident
as well as in the commercial development of this agent in the more than 300 articles that he has authored during
for the treatment of hemophilia in patients who have his career. However, the publications do not begin to
inhibitors. document completely all of the successes of the numerous
Throughout his career, Dr. Roberts has held leadership students, fellows, and faculty whom he has trained and
positions in professional societies nationally and interna- mentored. Those who have been privileged to work with
tionally and has served on editorial boards or as editor of him recount stories that reveal his warmth, humility, and
multiple journals. He has worked tirelessly with the Inter- wonderfully dry sense of humor. Dr. Roberts’ ability to
national Society on Thrombosis and Haemostasis, the nurtureandsustainlong-lastingrelationshipswithpatients,
American Society of Hematology, the World Federation of healthcareproviders,publicadvocacygroups,andgovern-
Hemophilia, the College of American Pathologists, and ment officials across multiple countries ensures that the
theNationalHemophiliaFoundation.Hehaschairedstudy advancesinclinicalcarethathehasdevelopedareavailable
sections and served on councils for the National Institutes topatients throughoutthenation andtheworld.
vi
Contributors
Victoria Afshani, MD Lisa N. Boggio, MS, MD
Hematologist/Medical Oncologist, Georgia Cancer Assistant Professor of Medicine and Pediatrics,
Specialists, Atlanta, Georgia Rush University Medical Center, Chicago, Illinois
Management of Bleeding Disorders in Pregnancy Hemophilia A and B
William C. Aird, MD Charles D. Bolan, MD
Associate Professor of Medicine, Harvard Medical Associate Professor of Medicine, Uniformed
School; Attending Physician and Chief, Division of ServicesUniversityoftheHealthSciencesF.Edward
Molecular and Vascular Medicine, Beth Israel He´bert School of Medicine; Staff Clinician and
Deaconess Medical Center, Boston, Massachusetts Hematology Fellowship Program Director,
Endothelium Hematology Branch, National Heart, Lung, and
Blood Institute, National Institutes of Health,
Barbara M. Alving, MD Bethesda, Maryland
Bethesda, Maryland Blood Component and Pharmacologic Therapy of
The Antiphospholipid Syndrome: Clinical Presentation, Hemostatic Disorders
Diagnosis, and Patient Management; Risk Factors for
Cardiovascular Disease and Arterial Thrombosis; Andrew K. Burroughs, MD
Management of Thrombophilia and Antiphospholipid Professor of Hepatology, Royal Free and University
Syndrome During Pregnancy College School of Medicine; Consultant Physician
and Hepatologist, Royal Free Hospital, London,
Jack E. Ansell, MD United Kingdom
Professor of Medicine, Boston University School of Hemostatic Alterations in Liver Disease and Liver
Medicine;ViceChairman,DepartmentofMedicine, Transplantation
Boston Medical Center, Boston, Massachusetts
Outpatient Anticoagulant Therapy Hugo ten Cate, MD
Department of Internal Medicine, University of
Kenneth Ataga, MD Maastricht Medical School, Maastricht, The
Assistant Professor, University of North Carolina at Netherlands
Chapel Hill School of Medicine; Attending The Cross-Talk of Inflammation and Coagulation in
Physician, University of North Carolina Hospitals, Infectious Disease and Their Roles in Disseminated
Chapel Hill, North Carolina Intravascular Coagulation
Hemostatic Aspects of Sickle Cell Disease
Richard Chang, MD
Richard C. Becker, MD Chief, Special Procedures, Department of
Professor of Medicine, Divisions of Cardiology Radiology, W. G. Magnuson Clinical Center,
and Hematology, Duke University School of National Institutes of Health, Bethesda, Maryland
Medicine; Director, Cardiovascular Thrombosis
Thrombosis Related to Venous Access Devices
Center, Duke Clinical Research Institute, Durham,
North Carolina Mary Cushman, MD, MSc
Hemostatic Aspects of Cardiovascular Medicine Associate Professor of Medicine, University of
VermontCollegeofMedicine;Director,Thrombosis
Peter C. Block, MD andHemostasisProgram,DivisionofHematology&
Professor of Medicine, Emory University School of Oncology, Fletcher Allen Health Care, Burlington,
Medicine; Attending Physician, Emory University Vermont
Hospitals and Grady Hospital, Atlanta, Georgia
Risk Factors for Cardiovascular Disease and Arterial
Atrial Septal Abnormalities and Cryptogenic Stroke Thrombosis
vii
Thomas G. DeLoughery, MD David L. Gillespie, MD
C
O Professor of Medicine and Pathology, Divisions of Professor of Surgery, Uniformed Services University
N
TR Hematology/Oncology and Laboratory Medicine, of the Health Sciences F. Edward He´bert School of
IBU Oregon Health and Science University, Portland, Medicine, Bethesda, Maryland; Chief and Program
T
O Oregon Director, Vascular Surgery, Walter Reed Army
R
S Medical Center, Washington, DC
Anticoagulation for Atrial Fibrillation and Prosthetic
Cardiac Valves Prevention, Diagnosis, and Treatment of the Postphlebitic
Syndrome
Jorge Di Paola, MD
Associate Professor of Pediatrics; Director, Samuel Z. Goldhaber, MD
Hemophilia Treatment Center, Carver College of Professor of Medicine, Harvard Medical School;
Medicine, University of Iowa, Iowa City, Iowa Director, Venous Thromboembolism Research
Group; Director, Anticoagulation Service; and Staff
Congenital and Acquired Disorders of Platelet Function
Cardiologist, Brigham and Women’s Hospital,
and Number
Boston, Massachusetts
Tieraona Low Dog, MD Deep Vein Thrombosis and Pulmonary Embolism
Clinical Assistant Professor, Department of
Medicine, University of Arizona College of David Green, MD, PhD
Medicine; Director of Education, Program in Professor of Medicine, Northwestern University
Integrative Medicine, University of Arizona Health Feinberg School of Medicine; Attending Physician,
Sciences Center, Tucson, Arizona Northwestern Memorial Hospital; Physician,
Northwestern Medical Faculty Foundation,
Dietary Supplements and Hemostasis
Chicago, Illinois
Miguel A. Escobar, MD Prevention and Treatment of Venous Thromboembolism
Assistant Professor of Medicine and Pediatrics, in Neurologic and Neurosurgical Patients
University of Texas Medical School at Houston;
Associate Medical Director, Gulf States Hemophilia Christine L. Hann, MD, PhD
& Thrombophilia Center, Houston, Texas Clinical Fellow, Department of Medical Oncology,
Johns Hopkins University School of Medicine,
Less Common Congenital Disorders of Hemostasis
Baltimore, Maryland
Bruce M. Ewenstein, MD, PhD Vena Caval Filters
Global Medical Director, Baxter BioScience,
Westlake Village, California John A. Heit, MD
Professor of Medicine, Mayo Clinic College of
Nonhemophilic Inhibitors of Coagulation
Medicine; Director, Coagulation Laboratories and
Andres Fernandez, MD Clinic, and Consultant, Division of Cardiovascular
Diseases, Mayo Clinic, Rochester, Minnesota
Postdoctoral Research Fellow, Neurology
Department, Columbia University College of Thrombophilia: Clinical and Laboratory Assessment and
Physicians and Surgeons, New York, New York Management
Hematologic Interventions for Acute Central Nervous
John R. Hess, MD
System Disease
Professor of Pathology and Medicine, University of
Charles W. Francis, MD Maryland School of Medicine, Baltimore, Maryland
Professor of Medicine and Pathology and Hemorrhage Control and Thrombosis Following
Laboratory Medicine, University of Rochester Severe Injury
School of Medicine and Dentistry, Rochester,
New York William R. Hiatt, MD
Professor of Medicine, University of Colorado
Antithrombotic Agents
School of Medicine, Denver, Colorado
James N. George, MD Peripheral Arterial Disease
George Lynn Cross Professor, Department of
Medicine, University of Oklahoma College of Christopher D. Hillyer, MD
Medicine, Oklahoma City, Oklahoma Professor, Department of Pathology and
Laboratory Medicine, and Director, Transfusion
Immune Thrombocytopenic Purpura
Medicine, Emory University School of Medicine;
Director, Medical, Blood Bank, Emory University
Hospital, Atlanta, Georgia
Therapeutic Apheresis
viii
John B. Holcomb, MD Craig S. Kitchens, MD
C
Adjunct Professor of Surgery, University of Texas Professor of Medicine, University of Florida; O
N
School of Medicine at San Antonio, San Antonio; Associate Chief of Staff, Malcom Randall Veterans TR
Trauma Consultant for the Surgeon General and Administration Medical Center, Gainesville, Florida IBU
T
Commander, U.S. Army Institute of Surgical O
The Consultative Process; Purpura and Other R
Research, Fort Sam Houston, Texas S
Hematovascular Disorders; Disseminated
Hemorrhage Control and Thrombosis Following Intravascular Coagulation; Venous Thromboses at
Severe Injury Unusual Sites; Surgery and Hemostasis
McDonald K. Horne, III, MD Harvey G. Klein, MD
Senior Clinical Investigator, Hematology Service, Adjunct Professor of Medicine and Pathology,
W.G.MagnusonClinicalCenter,NationalInstitutes Johns Hopkins, University School of Medicine;
of Health, Bethesda, Maryland Chief, Department of Transfusion Medicine, W. G.
Magnuson Clinical Center, National Institutes of
Thrombosis Related to Venous Access Devices
Health, Bethesda, Maryland
Mark R. Jackson, MD Blood Component and Pharmacologic Therapy of
AssistantClinicalProfessor, Department ofSurgery, Hemostatic Disorders
Columbia University College of Physicians and
Kiarash Kojouri, MD, MPH
Surgeons, New York, New York; Vascular Surgeon,
Assistant Professor of Medicine, University of
St. Francis Hospital, Greenville, South Carolina
Oklahoma College of Medicine, Oklahoma City,
Topical Hemostatic Agents for Localized Bleeding
Oklahoma
Shawn Jobe, MD, PhD Immune Thrombocytopenic Purpura
Fellow, Pediatric Hematology Oncology, Carver
Barbara A. Konkle, MD
College of Medicine, University of Iowa, Iowa City,
Professor of Medicine; Director, Penn
Iowa
Comprehensive Hemophilia and Thrombosis
Congenital and Acquired Disorders of Platelet Function Program, University of Pennsylvania, Philadelphia,
and Number Pennsylvania
Thrombotic Risk of Contraceptives and Other Hormonal
Eefje Jong, MD
Therapies
Resident, Department of Internal Medicine,
UniversityofAmsterdamAcademicMedicalCenter, Kendra Kubiak, MD
Amsterdam, The Netherlands
Clinical Instructor in Medicine, University of
The Cross-Talk of Inflammation and Coagulation in Virginia School of Medicine, Charlottesville,
Infectious Disease and Their Roles in Disseminated Virginia
Intravascular Coagulation Point-of-Care Testing for Hemostatic Disorders
Craig M. Kessler, MD Jody L. Kujovich, MD
Professor of Medicine and Pathology, Georgetown Clinical Assistant Professor of Medicine, Oregon
University School of Medicine; Chief, Coagulation Health & Science University School of Medicine;
Laboratory, Lombardi Comprehensive Cancer Physician, Northwest Cancer Specialists, Portland,
Center, Washington, DC Oregon
A Systematic Approach to the Bleeding Patient: Management of Thrombophilia and Antiphospholipid
Correlation of Clinical Symptoms and Signs with Syndrome During Pregnancy
LaboratoryTesting;HemophiliaAandB;Thrombocytosis:
Essential Thrombocythemia and Reactive Causes David J. Kuter, MD, DPhil
Assistant Professor of Medicine, Harvard Medical
Nushmia Khokhar, MD School, Boston; Associate Professor of Medicine,
Department of Internal Medicine, Washington Health Sciences and Technology, Massachusetts
Hospital Center/Georgetown University Hospital, Institute of Technology, Cambridge; Director of
Washington, DC Clinical Hematology, Massachusetts General
A Systematic Approach to the Bleeding Patient: Hospital, Boston, Massachusetts
Correlation of Clinical Symptoms and Signs with General Aspects of Thrombocytopenia, Platelet
Laboratory Testing Transfusions, and Thrombopoietic Growth Factors
ix
Carrie LaBelle, MD Merry Jennifer Markham, MD
C
O Medical Director, Compensation and Pension Fellow, Division of Hematology/Oncology,
N
TR Service, Veterans Health System, Malcom Randall University of Florida Department of Medicine,
IBU VA Medical Center, Gainesville, Florida Gainesville, Florida
T
O
R Disseminated Intravascular Coagulation Dietary Supplements and Hemostasis
S
Marcel Levi, MD Stephan A. Mayer, MD
Professor of Medicine, University of Amsterdam Associate Professor of Clinical Neurology and
School of Medicine; Chairman, Department of Neurosurgery, Columbia University College of
Medicine, University of Amsterdam Academic Physicians and Surgeons; Director, Neuro-ICU,
Medical Center, Amsterdam, The Netherlands NewYork–Presbyterian Hospital, New York,
New York
The Cross-Talk of Inflammation and Coagulation in
Infectious Disease and Their Roles in Disseminated Hematologic Interventions for Acute Central Nervous
Intravascular Coagulation System Disease
Miles B. Levin, MD Jan Jacques Michiels, MD, PhD
Chief Resident in Pathology, Montefiore Medical Professor, Department of Hematology, Blood
Center, Bronx, New York Coagulation and Vascular Research, University of
Antwerp Faculty of Medicine/University Hospital
The Antiphospholipid Syndrome: Clinical Presentation,
Antwerp, Antwerp, Belgium; Director, Department
Diagnosis, and Patient Management
of Hematology, Hemostasis and Thrombosis
Mark Levine, MD, MSc Research, Hemostasis and Thrombosis Science
Professor of Clinical Epidemiology and Biostatistics Center, Goodheart Institute and Foundation,
Rotterdam; Scientific Medical Advisor, Medical
and Medicine and Buffet Taylor Chair of Breast
Diagnostic Center, Rotterdam; and Project Leader,
Cancer Research, Michael G. DeGroote School of
Deep Vein Thrombosis and the Post-thrombotic
Medicine, McMaster University Faculty of Health
Sciences, Hamilton, Ontario, Canada Syndrome, Erasmus Medical Center, Rotterdam,
The Netherlands
Thrombosis and Cancer
Thrombocytosis: Essential Thrombocythemia and
Minetta Liu, MD Reactive Causes
Assistant Professor of Medicine and Oncology,
Joel L. Moake, MD
Lombardi Comprehensive Cancer Center,
Professor of Medicine, Baylor College of Medicine;
Georgetown University Hospital, Washington, DC
Associate Director, Biomedical Engineering
A Systematic Approach to the Bleeding Patient:
Laboratory, Rice University, Houston, Texas
Correlation of Clinical Symptoms and Signs with
Thrombotic Thrombocytopenic Purpura
Laboratory Testing
Richard Lottenberg, MD Thomas L. Ortel, MD, PhD
Associate Professor of Medicine and Pathology,
Professor of Medicine, Division of Hematology/
Duke University School of Medicine, Durham,
Oncology, University of Florida College of
North Carolina
Medicine, Gainesville, Florida
Anticoagulation in the Perioperative Period
Hemostatic Aspects of Sickle Cell Disease
Reagan W. Quan, MD
B. Gail Macik, MD
Fellow, Vascular Surgery, Uniformed Services
Associate Clinical Professor of Medicine and
University of the Health Sciences F. Edward He´bert
Pathology, University of Virginia School of
School of Medicine, Bethesda, Maryland, and
Medicine, Charlottesville, Virginia
WalterReedArmyMedicalCenter,Washington,DC
Point-of-Care Testing for Hemostatic Disorders
Prevention, Diagnosis, and Treatment of the Postphlebitic
Victor J. Marder, MD Syndrome
Professor of Medicine, Division of Hematology/
Jacob H. Rand, MD
Medical Oncology, David Geffen School of
Professor of Pathology and Medicine, Albert
Medicine at UCLA, Los Angeles, California
Einstein College of Medicine of Yeshiva University;
Thrombolytic Therapy Director of Hematology Laboratories, Montefiore
Medical Center, Bronx, New York
The Antiphospholipid Syndrome: Clinical Presentation,
Diagnosis, and Patient Management
x
Margaret E. Rick, MD Suman Sood, MD
C
Assistant Chief, Hematology Service, W. G. Instructor in Medicine, Division of Hematology- O
N
Magnuson Clinical Center, National Institutes of Oncology, University of Pennsylvania School of TR
Health, Bethesda, Maryland Medicine; Penn Comprehensive Hemophilia and IBU
T
Thrombosis Program, University of Pennsylvania O
von Willebrand Disease R
Health System, Philadelphia, Pennsylvania S
Frederick R. Rickles, MD, FACP Thrombotic Risk of Contraceptives and Other
Professor of Medicine, Pediatrics, and Hormonal Therapies
Pharmacology and Physiology,George Washington
University School of Medicine and Health Sciences, Steven Stein, MD
Washington, DC; Fellow, Center for Science and Adjunct Assistant Professor, Hemophilia and
Technology, Noblis, Falls Church, Virginia Thrombosis Program, University of Pennsylvania
Thrombosis and Cancer School of Medicine, Philadelphia,
Pennsylvania
Fred Rincon, MD Thrombotic Risk of Contraceptives and Other
Clinical Assistant in Neurology and Post-Doctoral Hormonal Therapies
ClinicalFellow,ColumbiaUniversityMedicalCenter,
New York, New York Michael B. Streiff, MD
Hematologic Interventions for Acute Central Nervous Assistant Professor of Medicine, Johns Hopkins
System Disease University School of Medicine; Medical Director,
Anticoagulation Management Service, Johns
Harold R. Roberts, MD Hopkins Medical Institutions, Baltimore, Maryland
Sarah Graham Kenan Distinguished Professor of
Vena Caval Filters
Medicine and Pathology, Department of Medicine,
Division of Hematology and Oncology, and Bundarika Suwanawiboon, MD
Carolina Cardiovascular Biology Center, University
Instructor, Division of Hematology, Department of
of North Carolina at Chapel Hill School of Medicine, Faculty of Medicine, Mahidal University/
Medicine; Director, Comprehensive Hemophilia
Siriraj Hospital, Bangkok, Thailand
Treatment Center, University of North Carolina
Anticoagulation in the Perioperative Period
Hospitals, Chapel Hill, North Carolina
Less Common Congenital Disorders of Hemostasis Eric C.M. van Gorp, MD
Staff Physician, Department of Internal Medicine,
Lewis J. Rubin, MD
Slotervaart Hospital, Amsterdam, The Netherlands
Professor of Medicine, University of California,
The Cross-Talk of Inflammation and Coagulation in
San Diego, School of Medicine, La Jolla, California
Infectious Disease and Their Roles in Disseminated
PulmonaryHypertension:ThromboticandNonthrombotic
Intravascular Coagulation
in Origin
Theodore E. Warkentin, MD
Marco Senzolo, MD
Professor, Department of Pathology and Molecular
Consultant Gastroenterologist/Hepatologist,
Medicine and Department of Medicine, Michael G.
GastroenterologyUnit,DepartmentofSurgicaland
DeGroote School of Medicine, McMaster University
Gastroenterological Sciences, University Hospital of Faculty of Health Sciences; Associate Head,
Padua, Padua, Italy
Transfusion Medicine, Hamilton Regional
Hemostatic Alterations in Liver Disease and Liver Laboratory Medicine Program; Hematologist,
Transplanatation Service of Clinical Hematology, Hamilton Health
Sciences, Hamilton General Hospital, Hamilton,
Stephanie Seremetis, MD Ontario, Canada
Global Medical Director, Hemostasis, NovoNordisk
Heparin-Induced Thrombocytopenia
A/S, Bagsvaerd, Denmark
Management of Bleeding Disorders in Pregnancy Ann B. Zimrin, MD
Assistant Professor of Medicine, University of
Chelsea A. Sheppard, MD Maryland School of Medicine/Marlene and Stewart
Transfusion Medicine Fellow, Department of Greenebaum Cancer Center, Baltimore, Maryland
Pathology and Laboratory Medicine, Emory
Hemorrhage Control and Thrombosis Following
University School of Medicine/Emory University
Severe Injury
Hospital, Atlanta, Georgia
Therapeutic Apheresis Marc Zumberg, MD
Associate Professor of Medicine, University of
Florida College of Medicine, Gainesville, Florida
Purpura and Other Hematovascular Disorders; Venous
Thromboses at Unusual Sites
xi
Preface
We editors were pleased by the enthusiasm and success von Willebrand disease, and heparin-induced thrombocy-
with which the first edition of Consultative Hemostasis topenia, as well as thrombotic thrombocytopenia purpura
and Thrombosis was met. Clearly, there is a niche for a and other disorders. Our second goal was to ensure a very
mid-sized textbook on hemostasis and thrombosis that strongintegrationamongthespecialtiesthatdealwithclin-
can authoritatively assist the busy consultant; thus, we are ical issues in thrombosis and hemostasis; these include
presenting an updated second edition. cardiology, neurology, oncology, obstetrics, and vascular
Much has happened since the first edition appeared in surgery. Accordingly, we tapped internationally renowned
early 2002. For example, the discovery of ADAMTS-13 authors writing on hemostatic and thrombotic complica-
andtheelucidationofitsroleinthromboticthrombocyto- tions associated with such conditions as a patent foraman
penic purpurawere just being accomplished when the first ovale, pulmonary hypertension, malignancy, indwelling
edition was published. catheters, trauma, and pregnancy.
The use of recombinant activated factor VII (rFVIIa) We are deeply grateful to our contributing authors, and
has expanded greatly beyond the original indication for we appreciate the colleagues who have given us support
which it was licensed. This is reviewed in detail in this andconstructivecriticismforthissecondedition.Wehope
new edition. We are now seeing the increased use of that this book will serve as an improved and useful guide
thrombin-specificinhibitors,andinitialstudiesofthepenta- forallwhoareinvolvedinprovidingconsultationandcare
saccharide fondaparinux in the treatment of heparin- for patients with hemostatic or thrombotic disorders.
inducedthrombocytopeniaarepromising.
As editors of the book, we have focused on two primary Craig S. Kitchens, MD
goals.Onewastoprovideupdatesonthe“corematerial”for
Barbara M. Alving, MD
hemostasis and thrombosis, with internationally renowned
experts writing chapters on deep vein thrombosis, pul- Craig M. Kessler, MD
monary embolus, hypercoagulability, thrombocytopenia,
xiii
Part I
General Information