E-Book Description
Hemostasis and Thrombosis Basic Principles and Clinical Practice 6th Edition PDF Free Download
Since publication of the First Edition in 1982, Hemostasis and Thrombosis has established itself as the pre-eminent book in the field of coagulation disorders. No other book is as inclusive in scope, with coverage of the field from the standpoint of both basic scientists and clinicians. This comprehensive resource details the essentials of bleeding and thrombotic disorders and the management of patients with these and related problems, and delivers the most up-to-date information on normal biochemistry and function of platelets or endothelial cells, as well as in-depth discussions of the pharmacology of anticoagulant, fibrinolytic, and hemostatic drugs.
NEW to the Sixth Edition…
• A new team of editors , each a leader in his field, assures you of fresh, authoritative perspectives.
• Full color throughout
• A companion website that offers full text online and an image bank.
• A new introductory section of chapters on basic sciences as related to the field
• Entirely new section on Hemostatic and Thrombotic Disorders Associated with Systemic Conditions includes material on pediatric patients, women’s health issues, cancer, sickle cell disease, and other groups.
• Overview chapters preceding each section address broad topics of general importance.
E-Book Details
Сategories: Medicine|Hematology
Hardcover : 1592 pages
ISBN-10 : 9781608319060
ISBN-13 : 978-1608319060
ASIN : 1608319067
Language: : English
Hardcover : 1592 pages
ISBN-10 : 9781608319060
ISBN-13 : 978-1608319060
ASIN : 1608319067
Language: : English
Table of Contents
Preface xv
From the Preface to the First Edition xv11
Acknowledgments xix
PART I :
I NTRODUCTION TO TH E F I E LD O F
H E MOSTASIS A N D TH ROM BOSIS
1. The Field of Hemostasis and Thrombosis:
Selected Translational Achievements 3
Victor J. Marder, William C. Aird, Joel S. Bennett, Sam Schulman,
and Gilbert C. White, II
2. Molecular Evolution of the Vertebrate Blood
Coagu lation System . 1 1
Marianne A. Grant and William C. Aird
3. Molecular and Clin ical Genetics . 26
Lesley Everett, Karl Desch, and David Ginsburg
4. Coagu lation Enzymology . 4 1
Sriram Krishnaswamy
5. Cell Biology, Protein Processi ng, and Cell
Signaling . 52
Randal J. Kaufman and Laura Popolo
6. Overview of the I m m u ne System 66
Andrew H. Lichtman
7. Biostatistics and Epidemiology: The Basic
Science of Clinical Research . 87
Kerry L. Lee
PART I I :
BASIC PRI NCIPLES O F H E MOSTASIS
AND TH ROMBOSIS
S ECTI O N A: COAG U LATI O N A N D F I B R I NOLYS I S
8. Overview of Basic Coagulation and
Fibrinolysis . 1 03
Gilbert C. White, II, Victor J. Marder, Sam Schulman,
William C. Aird, and Joel S. Bennett
9. The Blood Coagu lation Factors and I n h i b itors:
Their Primary Structure, Com plementary
DNAs, Genes, and Expression 1 1 0
Dominic W. Chung, Wenfeng Xu, and Earl W. Davie
1 0. Genetics of Coagu lation . 1 46
Rodney M. Camire and Paris Margaritis
1 1 . Tissue Factor and the I n itiation and Regulation
(TF PI) of Coagu lation . 1 63
James H. Morrissey and George J. Broze, Jr.
1 2. Factor V and Factor VI I I 1 79
Randal J. Kaufman, Philip J. Fay, Laura Popolo, and Thomas L. Orte!
1 3. Structure and Function of von Willebrand
Factor . 1 97
Sandra L. Haberichter and Robert R. Montgomery
CONT E NTS
1 4. Structure and Function of Vitamin KDependent
Coagulant and Anticoagu lant
Proteins . 208
Kanagasabai Vadivel, Amy E. Schmidt, Victor J. Marder,
Sriram Krishnaswamy, and S. Paul Bajaj
1 5A. Factor XI 233
Peter N. Walsh and David Gailani
1 5B. The Factor XI I-Driven Plasma Contact System 242
Thomas Renne
1 6. Fibrinogen Structure and Function 254
John W. Weisel and Carl-Erik H. Dempfle
1 7. Factor X I I I . 2 72
Cedric Duval, Helen Philippon, and Robert A.S. Ariens
1 8. Antithrombin and the Serpin Fam i ly 286
Susan C. Bock
1 9. Protein C, Protein S, Throm bomodulin, and
the Endothelial Protein C Receptor Pathways 300
Laurent 0. Mosnier and John H. Griffin
20. Plasminogen Activation and Regulation of
Fibrinolysis 3 1 4
Nicola J. Mutch and Nuala A. Booth
2 1 . I n h i bitors of Fibrinolysis . 334
Bjorn Wiman and Anna Agren
S ECTI O N B: PLATELET B I O LOGY
22. Overview of Mega ka ryocyte and Platelet
Biology 341
Joel S. Bennett
23. Platelet Production: Cellular and Molecular
Regulation . 349
Elisabeth Cramer Borde and William Vainchenker
24. Megakaryocyte Structure and Platelet
Biogenesis . 365
Joseph E. Italiano, Jr. and John H. Hartwig
25. Th rombopoietin 3 73
Kenneth Kaushansky and Ian Hitchcock
26A. Major Platelet G lycoproteins: Platelet
G lycoprotei n lb-IX-V 382
Michael C. Berndt and Robert K. Andrews
26B. Major Platelet G lycoproteins: I NTEG R I N
a11b3 {G P l l b – l l la) . 386
Feng Ye and Mark H. Ginsberg
2 7. Platelet G lycoprotein Polymorphisms and
Relationship to Function and l m m unogenicity 393
Thomas J . Kunicki and Diane J . Nugent
28. The Basis for Platelet Adhesion . 400
Brian Savage and Zaverio M. Ruggeri
29. Shear Effects on Platelets and the Vessel Wall
in the Pathogenesis of Atherothrombosis 4 1 0
David L. Bark, Jr., Warwick S. Nesbitt, Angus K.T. Wong,
and Shaun P. Jackson
30. Platelet Collagen Receptors 420
Stephen P. Watson, Richard W. Farndale, Masaaki Moroi,
and Stephanie M. Jung
xxi
xx ii CONTENTS
3 1 . l ntegri n a11bA and Platelet Aggregation . 43 1
Ana Kasirer-Friede, Kamila Bledzka, Jun Qin, Edward F. Plow,
and Sanford J. Shattil
32. Molecular Basis for Platelet Secretion . 441
Shaojing Ye and Sidney W. Whiteheart
33. Platelet Signal Transduction . 449
Charles S. Abrams and Lawrence F. Brass
34. Platelet Prostanoid Metabolism 462
Tilo Grosser and Garret A. Fitzgerald
35. The Role of Platelets in Blood Coagu lation . 468
Peter N. Walsh
36. Blood-Borne M icroparticles 475
Jose A. L6pez and Swaminathan Murugappan
S ECTI O N C: VASCU LAR B I O LOGY
3 7. Overview of Vascular Biology 493
William C. Aird
38. Development of the Vascu latu re, Angiogenesis,
and Lym phangiogenesis . 498
Aernout Luttun and Peter Carmeliet
39. Rheology and Vessel Wa ll Stress 5 1 6
Larry V. Mcintire, Juan M. Jimenez, Suzanne G. Eskin,
and Peter F. Davies
40. N itric Oxide: The Vascular Biology of
N itric Oxide and N itric Oxide Synthases 536
Bradley A. Maron and Thomas Michel
4 1 . Blood Vessel Wall in Health and Disease . 549
Keith R. Brunt, Sonya Hui, Jagdish Butany, and Mansoor Husain
42. Endothelial Cell Function 560
William C. Aird
43. Role of the Endotheli u m in Hemostasis . 569
Michael J. Krisinger and Edward M. Conway
44. Coagu lation-I ndependent Signaling of the
Extri nsic Coagu lation Pathway . 5 79
Wolfram Ruf
45. Heparin and Vascular Proteoglycans 585
Douglas M. Tollefsen and Lijuan Zhang
46. Endothelial Cell-Blood Cell I nteractions . 598
Klaus Ley
47. Leukocyte Function and Contribution
to Disease . 6 1 4
Edith Van de Vijver and Taco W. Kuijpers
48. Laboratory Markers of Endothelial Cell
Activation and Dysfunction 623
Eduard Shantsila, Gregory Y.H. Lip, and Robert P. Hebbel
PART I l l :
DISORDERS OF COAG U LATION,
PLATELETS, AND VESSEL WALL
S ECTI O N A: B L E E D I N G DISORDERS O F COAG U LATI O N
A N D F I B R I N OLYS I S
49. Overview of Inherited Hemorrhagic Disorders . 641
Gilbert C. White, II, Harold R. Roberts, and Victor J. Marder
50. Differential Diagnosis
of the Bleedi ng Patient 648
Jill M. Johnsen and Barbara A. Konkle
5 1 . Cli nical Manifestations and Therapy
of I nherited and Acqu i red Hemophilia . 659
Edward G.D. Tuddenham, Peter W. Collins, and T.T. Yee
52. von Willebrand Disease: Diagnosis,
Classification, and Treatment 670
J. Evan Sadler and David Lillicrap
53. Rare Inherited Coagulation Disorders
Other than Hemophilia . 684
Pier Mannuccio Mannucci, Flora Peyvandi, and Massimo Franchini
54. Afibri nogenemias and Dysfibrinogenemias 693
Dennis K. Galanakis
55. Fam i lial M u ltiple Coagu lation Factor
Deficiencies 709
Bin Zhang and David Ginsburg
56. Cli nical Disorders of Fibrinolysis 7 1 5
Daphne B. Stewart, Charles W. Francis, and Victor J. Marder
5 7. Acqu i red Disorders of Coagu lation:
The Immune Coagulopathies . 723
Charles R.M. Hay
58. Laboratory Markers of Activated Coagu lation
and Its Regulation 738
Kenneth A. Bauer and Helen A. Ireland
S ECTI O N B: ACQ U I RE D A N D I N H ERITED P LATELET
DISORDERS
59. Overview of Acqu i red and I n herited Clinical
Platelet Disorders 75 1
Joel S. Bennett
60. Differential Diagnosis of Th rombocytopenia . 757
Joel S. Bennett and Charles S. Abrams
6 1 . I m m u ne Th rombocytopenia and
Posttransfusion Purpura . 763
Howard A. Liebman, Douglas B. Cines, and James B. Busse!
62. Drug- I nduced Thrombocytopenia . 776
Richard H. Aster and James N. George
63. I nherited Thrombocytopenias 785
Paquita Nurden and Alan T. Nurden
64. Acqu i red Nonimmune Th rombocytopenia . 796
Andreas Greinacher and Theodore E. Warkentin
65. I n herited Disorders of Platelet Fu nction . 805
Joel S. Bennett and A. Koneti Rao
66. Acqu i red Disorders of Platelet Fu nction . 820
A. Koneti Rao and Joel S. Bennett
67. Laboratory Markers of Platelet Activation . 829
Paul Harrison and Alan D. Michelson
S ECTI O N C: P R I M ARY DISORDERS OF BLOOD VESS E LS
68. Overview of Pri mary Disorders of Blood
Vessels 841
William C. Aird
69. Disorders of Connective Tissue
and Extracellular Matrix . 842
Fransiska Malfait and Anne De Paepe
70. Hereditary Hemorrhagic Telangiectasia . 855
Michelle Letarte and Claire L. Shovlin
71 . Arteriovenous Malformations and
Hemangiomas and Cerebral Cavernous
Malformations . 865
Kevin J. Whitehead, Douglas A. Marchuk, and Mark L. Kahn
72. Aneurysms (Large and Small Arteries) . 870
Carlos E. Sanchez, Sangita Kapur, and Neal L. Weintraub
73. l ntracranial Hemorrhage and Subarachnoid
Hemorrhage . 882
Timolaos Rizos and Thorsten Steiner
74. Retinopathy . 893
Jing Chen, Andreas Stahl, and Lois E. H. Smith
S ECTI O N D: T H E RAPY OF BLEEDI N G D I SORDERS
75. Overview of Therapy of Bleed ing Disorders 903
Gilbert C. White, II, Sam Schulman, and Victor J. Marder
76. Management of Acute Hemorrhage . 906
Micah J. Mooberry, Rommel P. Lu, and Nigel S. Key
77. Therapy with Antifi brinolytic Agents 9 1 8
Daphne B . Stewart and Victor J. Marder
78. Platelets, Plasma, and Specia lized Coagu lation
Concentrate Therapies 93 1
Thomas S. Kickier
79. New Approaches for the Therapy of Bleedi ng
Disorders, I ncluding Gene Therapy 942
Gilbert C. White, II and Harold R. Roberts
• PART IV:
ll’lll TH ROM BOTIC DISORDERS
S ECTI O N A: V E N O U S THROM BOTIC D I S O R D E RS
80. Overview of Venous Thromboembolism . 9 5 7
Sam Schulman, Victor J . Marder, and Gilbert C . White, I I
8 1 . Th rombophilia Genetics . 962
Joseph Emmerich, Martine Aiach, and Pierre-Emmanuel Morange
82. Epidemiology and Risk Factors for Venous
Th romboembolism . 9 73
John A. Heit
83. Clinical Man ifestations and Diagnosis of
Venous Thromboembolism 977
Henri Bounameaux a n d Arnaud Perrier
84. Venous Thrombosis in U n usual Sites 995
Victor J. Marder, Hylton V. Joffe, and Sam Schulman
85. Sequelae of Venous Thromboembolic Disease:
Postthrom botic Syndrome and Chronic
Th romboembolic Pulmonary Hypertension . 1 009
Vittorio Pengo and Susan R. Kahn
86. Prevention of Venous Thromboembolic
Disease 1 0 1 9
Menno V. Huisman, Sharif Pasha, Patrick Mismetti, and Ajay Kakkar
87. Treatment of Venous Thromboembolic
Disease 1 03 5
Sam Schulman, Jeffrey Ginsberg, and James Douketis
S ECTI O N B: ARTERIAL TH ROM BOTIC DISORDERS
88. Overview of Arteria l Thrombotic Disorders . 1 043
Sam Schulman and Victor J. Marder
89. Pathophysiology of Atherothrombosis . 1 047
Peter Libby, Paul M. Ridker, and Kevin Croce
90. Acute Coronary Syndromes: Manifestations
and Management 1 064
John Paul Vavalle, John H. Alexander, and Matthew T. Roe
CONTENTS xxiii
9 1 . Pathophysiology of Thromboembolic
Stroke 1 087
Erner R. McGrath and Martin J. O’Donnell
92. Prevention of Thromboembolic Stroke
in Patients with Atrial Fibrillation, Valvu la r
Heart Disease, or Cardiomyopathy 1 098
Thomas L. Kawano and Jonathan L. Halperin
93. lschemic Stroke: Acute Thrombolytic Therapy,
Antithrombotic Approaches, and General
Management 1 1 1 3
Gregory J. Del Zoppo and Jeffrey L. Saver
94. Medical and S u rgical Treatment of Peri pheral
Arterial Disease . 1 1 34
Philip Joseph, Saurabh Kalra, and Sonia Anand
95. U n usual Sites of Arterial Occlusion 1 1 5 5
Philip A. Kalra, Jon G. Moss, and Peter Wiedemann
96. Pathogenesis and Treatment of
Biomaterial-Associated Thrombosis 1 1 6 5
Maud B. Gorbet and Michael V. Sefton
S ECTI O N C: CO M P LEX THROM BOTIC DISORDERS
9 7. Overview of Complex Thrombohemorrhagic
Disorders . 1 1 73
Theodore E. Warkentin and Victor J. Marder
98. Consumptive Thrombohemorrhagic
Disorders . 1 1 78
Marcel Levi, Donald I. Feinstein, Robert W. Colman,
and Victor J. Marder
99. The Throm botic Thrombocytopenic Purpura
and Hemolytic U remic Syndromes . 1 1 96
James N. George
1 00. Vascu litides . 1 205
James R. Stone and John H. Stone
1 0 1 . The Anti phospholipid Syndrome . 1 2 1 6
Jacob H . Rand and Lucia R. Wolgast
1 02. Myeloproliferative Neoplasms and
Th rombohemorrhagic Complications 1 2 32
Ayalew Tefferi
1 03. Thrombotic and Hemorrhagic Complications
Associated with Stem Cell Transplantation . 1 244
Yona Nadir and Benjamin Brenner
S E CTI O N D: T H E RAPEUTIC AGE NTS
1 04. Overview of Therapeutic Agents Used
in Th rombotic Disorders 1 2 53
Sam Schulman and Victor J. Marder
1 0 5. Pharmacology and Mode of Action of Heparin
and G lycosam i noglycans . 1 2 58
Meyer Michel Samama, Jeanine M. Walenga, Evi Kalodiki,
and Jawed Fareed
1 06. Vitamin K Antagonists: Biochemistry,
Pharmacology, and Management 1 26 7
Nirmish Shah, Thomas L. Orte!, and Sam Schulman
1 0 7. Hemorrhagic Complications
of Anticoagulation . 1 2 8 1
Sam Schulman and Mark A . Crowther
1 08. Hepari n – I nduced Thrombocytopenia . 1 292
Theodore E. Warkentin and Andreas Greinacher
xxiv CONTENTS
1 09. Coumari n – l nduced Skin Necrosis and Venous
Limb Gangrene 1 308
Theodore E. Warkentin
1 1 0. New Antithrombotic Drugs . 1 3 1 8
Jeffrey I . Weitz and Peter L. Gross
1 1 1 . Anti platelet Therapy . 1 33 7
Carlo Patrono
1 1 2 . Fou ndations of Thrombolytic Therapy 1 349
Victor J. Marder and Daphne B. Stewart
1 1 3. Anticoagulant and Procoagulant Protei ns
in the Animal Ki ngdom . 1 36 1
Angelina Wei Ling Tan, Shiyang Kwong, and R. Manjunatha Kini
1 1 4. Mechanical Devices and Embolectomy . 1 3 70
Herve Decousus, Patrick Mismetti, and Nicolas Meneveau
PART V:
HEMOSTATIC AND TH ROMBOTIC
DISORDERS ASSOCIATED WITH
SYSTEM I C CON D ITIONS
1 1 5. Overview of Special Clinical G roups . 1 3 79
Victor J. Marder and Joel S. Bennett
1 1 6. Venous Thromboembolism d u ri ng Pregnancy
and with Hormonal Contraceptives 1 380
Saskia Middeldorp, Mathilde Nijkeuter, and Michie! Coppens
1 1 7. Gestational Vascular Complications 1 393
Benjamin Brenner, Anat Aharon, and Jean-Christophe Gris
1 1 8. Maternal Hemostatic Disorders and Obstetric
Hemorrhage 1 402
Philippe De Moerloose, Franoise Boehlen, and Jean-Christophe Gris
1 1 9. Developmental Hemostasis . 1 4 1 1
Paul Monagle, Vera Ignjatovic, and Leslie Roy Berry
1 20. Bleeding in Neonates and C h i ldren 1 424
Anthony K. C. Chan, Elizabeth A. Chalmers, Sam Schulman,
and Alan D. Michelson
1 2 1 . Venous and Arterial Thromboembolism
i n Children 1 434
Paul Monagle, M. Patricia Massicotte, and Anthony K. Chan
1 2 2. Thrombotic Complications of Sickle
Cell Disease . 1 445
Kenneth I. Ataga and Elliott P. Vichinsky
1 2 3. Th rombohemorrhagic Complications
of Sepsis . 1 454
Marcel Levi
1 24. Hemorrhagic Fevers: Epidemiology,
Pathophysiology, and Clin ical Features . 1 463
L. Gayani Tillekeratne, Jennifer Cohn, and Hillary Dunlevy
1 2 5. Cancer- Related Throm bosis . 1 468
Anna Falanga, Agnes Y. Y. Lee, and Frederick R. Rickles
1 26. Hemostatic Cha llenges in Liver Disease 1 48 1
Charles S . Eby and Stephen H. Caldwell
1 2 7. Hemostatic Abnormalities in Renal D isease . 1 49 1
Miriam Galbusera, Giuseppe Remuzzi, and Patrizia Ondei
1 28. M u ltitrauma Derangements in Hemostasis
and Thrombosis . 1 498
Diane A. Schwartz, Lucy Z. Kornblith, John B. Holcomb,
and Mitchell Jay Cohen
1 2 9. Blood Management in the Cardiovascular
Surgical Patient . 1 504
Lawrence Tim Goodnough and L. Henry Edmunds, Jr.
1 30. Th rombohemorrhagic Events in the Elderly:
Special Considerations . 1 5 1 5
Isabelle Mahe and Ludovic Dronet
Index 1 523
Hemostasis and Thrombosis Basic Principles and Clinical Practice 6th Edition PDF Ebook
From the Preface to the First Edition xv11
Acknowledgments xix
PART I :
I NTRODUCTION TO TH E F I E LD O F
H E MOSTASIS A N D TH ROM BOSIS
1. The Field of Hemostasis and Thrombosis:
Selected Translational Achievements 3
Victor J. Marder, William C. Aird, Joel S. Bennett, Sam Schulman,
and Gilbert C. White, II
2. Molecular Evolution of the Vertebrate Blood
Coagu lation System . 1 1
Marianne A. Grant and William C. Aird
3. Molecular and Clin ical Genetics . 26
Lesley Everett, Karl Desch, and David Ginsburg
4. Coagu lation Enzymology . 4 1
Sriram Krishnaswamy
5. Cell Biology, Protein Processi ng, and Cell
Signaling . 52
Randal J. Kaufman and Laura Popolo
6. Overview of the I m m u ne System 66
Andrew H. Lichtman
7. Biostatistics and Epidemiology: The Basic
Science of Clinical Research . 87
Kerry L. Lee
PART I I :
BASIC PRI NCIPLES O F H E MOSTASIS
AND TH ROMBOSIS
S ECTI O N A: COAG U LATI O N A N D F I B R I NOLYS I S
8. Overview of Basic Coagulation and
Fibrinolysis . 1 03
Gilbert C. White, II, Victor J. Marder, Sam Schulman,
William C. Aird, and Joel S. Bennett
9. The Blood Coagu lation Factors and I n h i b itors:
Their Primary Structure, Com plementary
DNAs, Genes, and Expression 1 1 0
Dominic W. Chung, Wenfeng Xu, and Earl W. Davie
1 0. Genetics of Coagu lation . 1 46
Rodney M. Camire and Paris Margaritis
1 1 . Tissue Factor and the I n itiation and Regulation
(TF PI) of Coagu lation . 1 63
James H. Morrissey and George J. Broze, Jr.
1 2. Factor V and Factor VI I I 1 79
Randal J. Kaufman, Philip J. Fay, Laura Popolo, and Thomas L. Orte!
1 3. Structure and Function of von Willebrand
Factor . 1 97
Sandra L. Haberichter and Robert R. Montgomery
CONT E NTS
1 4. Structure and Function of Vitamin KDependent
Coagulant and Anticoagu lant
Proteins . 208
Kanagasabai Vadivel, Amy E. Schmidt, Victor J. Marder,
Sriram Krishnaswamy, and S. Paul Bajaj
1 5A. Factor XI 233
Peter N. Walsh and David Gailani
1 5B. The Factor XI I-Driven Plasma Contact System 242
Thomas Renne
1 6. Fibrinogen Structure and Function 254
John W. Weisel and Carl-Erik H. Dempfle
1 7. Factor X I I I . 2 72
Cedric Duval, Helen Philippon, and Robert A.S. Ariens
1 8. Antithrombin and the Serpin Fam i ly 286
Susan C. Bock
1 9. Protein C, Protein S, Throm bomodulin, and
the Endothelial Protein C Receptor Pathways 300
Laurent 0. Mosnier and John H. Griffin
20. Plasminogen Activation and Regulation of
Fibrinolysis 3 1 4
Nicola J. Mutch and Nuala A. Booth
2 1 . I n h i bitors of Fibrinolysis . 334
Bjorn Wiman and Anna Agren
S ECTI O N B: PLATELET B I O LOGY
22. Overview of Mega ka ryocyte and Platelet
Biology 341
Joel S. Bennett
23. Platelet Production: Cellular and Molecular
Regulation . 349
Elisabeth Cramer Borde and William Vainchenker
24. Megakaryocyte Structure and Platelet
Biogenesis . 365
Joseph E. Italiano, Jr. and John H. Hartwig
25. Th rombopoietin 3 73
Kenneth Kaushansky and Ian Hitchcock
26A. Major Platelet G lycoproteins: Platelet
G lycoprotei n lb-IX-V 382
Michael C. Berndt and Robert K. Andrews
26B. Major Platelet G lycoproteins: I NTEG R I N
a11b3 {G P l l b – l l la) . 386
Feng Ye and Mark H. Ginsberg
2 7. Platelet G lycoprotein Polymorphisms and
Relationship to Function and l m m unogenicity 393
Thomas J . Kunicki and Diane J . Nugent
28. The Basis for Platelet Adhesion . 400
Brian Savage and Zaverio M. Ruggeri
29. Shear Effects on Platelets and the Vessel Wall
in the Pathogenesis of Atherothrombosis 4 1 0
David L. Bark, Jr., Warwick S. Nesbitt, Angus K.T. Wong,
and Shaun P. Jackson
30. Platelet Collagen Receptors 420
Stephen P. Watson, Richard W. Farndale, Masaaki Moroi,
and Stephanie M. Jung
xxi
xx ii CONTENTS
3 1 . l ntegri n a11bA and Platelet Aggregation . 43 1
Ana Kasirer-Friede, Kamila Bledzka, Jun Qin, Edward F. Plow,
and Sanford J. Shattil
32. Molecular Basis for Platelet Secretion . 441
Shaojing Ye and Sidney W. Whiteheart
33. Platelet Signal Transduction . 449
Charles S. Abrams and Lawrence F. Brass
34. Platelet Prostanoid Metabolism 462
Tilo Grosser and Garret A. Fitzgerald
35. The Role of Platelets in Blood Coagu lation . 468
Peter N. Walsh
36. Blood-Borne M icroparticles 475
Jose A. L6pez and Swaminathan Murugappan
S ECTI O N C: VASCU LAR B I O LOGY
3 7. Overview of Vascular Biology 493
William C. Aird
38. Development of the Vascu latu re, Angiogenesis,
and Lym phangiogenesis . 498
Aernout Luttun and Peter Carmeliet
39. Rheology and Vessel Wa ll Stress 5 1 6
Larry V. Mcintire, Juan M. Jimenez, Suzanne G. Eskin,
and Peter F. Davies
40. N itric Oxide: The Vascular Biology of
N itric Oxide and N itric Oxide Synthases 536
Bradley A. Maron and Thomas Michel
4 1 . Blood Vessel Wall in Health and Disease . 549
Keith R. Brunt, Sonya Hui, Jagdish Butany, and Mansoor Husain
42. Endothelial Cell Function 560
William C. Aird
43. Role of the Endotheli u m in Hemostasis . 569
Michael J. Krisinger and Edward M. Conway
44. Coagu lation-I ndependent Signaling of the
Extri nsic Coagu lation Pathway . 5 79
Wolfram Ruf
45. Heparin and Vascular Proteoglycans 585
Douglas M. Tollefsen and Lijuan Zhang
46. Endothelial Cell-Blood Cell I nteractions . 598
Klaus Ley
47. Leukocyte Function and Contribution
to Disease . 6 1 4
Edith Van de Vijver and Taco W. Kuijpers
48. Laboratory Markers of Endothelial Cell
Activation and Dysfunction 623
Eduard Shantsila, Gregory Y.H. Lip, and Robert P. Hebbel
PART I l l :
DISORDERS OF COAG U LATION,
PLATELETS, AND VESSEL WALL
S ECTI O N A: B L E E D I N G DISORDERS O F COAG U LATI O N
A N D F I B R I N OLYS I S
49. Overview of Inherited Hemorrhagic Disorders . 641
Gilbert C. White, II, Harold R. Roberts, and Victor J. Marder
50. Differential Diagnosis
of the Bleedi ng Patient 648
Jill M. Johnsen and Barbara A. Konkle
5 1 . Cli nical Manifestations and Therapy
of I nherited and Acqu i red Hemophilia . 659
Edward G.D. Tuddenham, Peter W. Collins, and T.T. Yee
52. von Willebrand Disease: Diagnosis,
Classification, and Treatment 670
J. Evan Sadler and David Lillicrap
53. Rare Inherited Coagulation Disorders
Other than Hemophilia . 684
Pier Mannuccio Mannucci, Flora Peyvandi, and Massimo Franchini
54. Afibri nogenemias and Dysfibrinogenemias 693
Dennis K. Galanakis
55. Fam i lial M u ltiple Coagu lation Factor
Deficiencies 709
Bin Zhang and David Ginsburg
56. Cli nical Disorders of Fibrinolysis 7 1 5
Daphne B. Stewart, Charles W. Francis, and Victor J. Marder
5 7. Acqu i red Disorders of Coagu lation:
The Immune Coagulopathies . 723
Charles R.M. Hay
58. Laboratory Markers of Activated Coagu lation
and Its Regulation 738
Kenneth A. Bauer and Helen A. Ireland
S ECTI O N B: ACQ U I RE D A N D I N H ERITED P LATELET
DISORDERS
59. Overview of Acqu i red and I n herited Clinical
Platelet Disorders 75 1
Joel S. Bennett
60. Differential Diagnosis of Th rombocytopenia . 757
Joel S. Bennett and Charles S. Abrams
6 1 . I m m u ne Th rombocytopenia and
Posttransfusion Purpura . 763
Howard A. Liebman, Douglas B. Cines, and James B. Busse!
62. Drug- I nduced Thrombocytopenia . 776
Richard H. Aster and James N. George
63. I nherited Thrombocytopenias 785
Paquita Nurden and Alan T. Nurden
64. Acqu i red Nonimmune Th rombocytopenia . 796
Andreas Greinacher and Theodore E. Warkentin
65. I n herited Disorders of Platelet Fu nction . 805
Joel S. Bennett and A. Koneti Rao
66. Acqu i red Disorders of Platelet Fu nction . 820
A. Koneti Rao and Joel S. Bennett
67. Laboratory Markers of Platelet Activation . 829
Paul Harrison and Alan D. Michelson
S ECTI O N C: P R I M ARY DISORDERS OF BLOOD VESS E LS
68. Overview of Pri mary Disorders of Blood
Vessels 841
William C. Aird
69. Disorders of Connective Tissue
and Extracellular Matrix . 842
Fransiska Malfait and Anne De Paepe
70. Hereditary Hemorrhagic Telangiectasia . 855
Michelle Letarte and Claire L. Shovlin
71 . Arteriovenous Malformations and
Hemangiomas and Cerebral Cavernous
Malformations . 865
Kevin J. Whitehead, Douglas A. Marchuk, and Mark L. Kahn
72. Aneurysms (Large and Small Arteries) . 870
Carlos E. Sanchez, Sangita Kapur, and Neal L. Weintraub
73. l ntracranial Hemorrhage and Subarachnoid
Hemorrhage . 882
Timolaos Rizos and Thorsten Steiner
74. Retinopathy . 893
Jing Chen, Andreas Stahl, and Lois E. H. Smith
S ECTI O N D: T H E RAPY OF BLEEDI N G D I SORDERS
75. Overview of Therapy of Bleed ing Disorders 903
Gilbert C. White, II, Sam Schulman, and Victor J. Marder
76. Management of Acute Hemorrhage . 906
Micah J. Mooberry, Rommel P. Lu, and Nigel S. Key
77. Therapy with Antifi brinolytic Agents 9 1 8
Daphne B . Stewart and Victor J. Marder
78. Platelets, Plasma, and Specia lized Coagu lation
Concentrate Therapies 93 1
Thomas S. Kickier
79. New Approaches for the Therapy of Bleedi ng
Disorders, I ncluding Gene Therapy 942
Gilbert C. White, II and Harold R. Roberts
• PART IV:
ll’lll TH ROM BOTIC DISORDERS
S ECTI O N A: V E N O U S THROM BOTIC D I S O R D E RS
80. Overview of Venous Thromboembolism . 9 5 7
Sam Schulman, Victor J . Marder, and Gilbert C . White, I I
8 1 . Th rombophilia Genetics . 962
Joseph Emmerich, Martine Aiach, and Pierre-Emmanuel Morange
82. Epidemiology and Risk Factors for Venous
Th romboembolism . 9 73
John A. Heit
83. Clinical Man ifestations and Diagnosis of
Venous Thromboembolism 977
Henri Bounameaux a n d Arnaud Perrier
84. Venous Thrombosis in U n usual Sites 995
Victor J. Marder, Hylton V. Joffe, and Sam Schulman
85. Sequelae of Venous Thromboembolic Disease:
Postthrom botic Syndrome and Chronic
Th romboembolic Pulmonary Hypertension . 1 009
Vittorio Pengo and Susan R. Kahn
86. Prevention of Venous Thromboembolic
Disease 1 0 1 9
Menno V. Huisman, Sharif Pasha, Patrick Mismetti, and Ajay Kakkar
87. Treatment of Venous Thromboembolic
Disease 1 03 5
Sam Schulman, Jeffrey Ginsberg, and James Douketis
S ECTI O N B: ARTERIAL TH ROM BOTIC DISORDERS
88. Overview of Arteria l Thrombotic Disorders . 1 043
Sam Schulman and Victor J. Marder
89. Pathophysiology of Atherothrombosis . 1 047
Peter Libby, Paul M. Ridker, and Kevin Croce
90. Acute Coronary Syndromes: Manifestations
and Management 1 064
John Paul Vavalle, John H. Alexander, and Matthew T. Roe
CONTENTS xxiii
9 1 . Pathophysiology of Thromboembolic
Stroke 1 087
Erner R. McGrath and Martin J. O’Donnell
92. Prevention of Thromboembolic Stroke
in Patients with Atrial Fibrillation, Valvu la r
Heart Disease, or Cardiomyopathy 1 098
Thomas L. Kawano and Jonathan L. Halperin
93. lschemic Stroke: Acute Thrombolytic Therapy,
Antithrombotic Approaches, and General
Management 1 1 1 3
Gregory J. Del Zoppo and Jeffrey L. Saver
94. Medical and S u rgical Treatment of Peri pheral
Arterial Disease . 1 1 34
Philip Joseph, Saurabh Kalra, and Sonia Anand
95. U n usual Sites of Arterial Occlusion 1 1 5 5
Philip A. Kalra, Jon G. Moss, and Peter Wiedemann
96. Pathogenesis and Treatment of
Biomaterial-Associated Thrombosis 1 1 6 5
Maud B. Gorbet and Michael V. Sefton
S ECTI O N C: CO M P LEX THROM BOTIC DISORDERS
9 7. Overview of Complex Thrombohemorrhagic
Disorders . 1 1 73
Theodore E. Warkentin and Victor J. Marder
98. Consumptive Thrombohemorrhagic
Disorders . 1 1 78
Marcel Levi, Donald I. Feinstein, Robert W. Colman,
and Victor J. Marder
99. The Throm botic Thrombocytopenic Purpura
and Hemolytic U remic Syndromes . 1 1 96
James N. George
1 00. Vascu litides . 1 205
James R. Stone and John H. Stone
1 0 1 . The Anti phospholipid Syndrome . 1 2 1 6
Jacob H . Rand and Lucia R. Wolgast
1 02. Myeloproliferative Neoplasms and
Th rombohemorrhagic Complications 1 2 32
Ayalew Tefferi
1 03. Thrombotic and Hemorrhagic Complications
Associated with Stem Cell Transplantation . 1 244
Yona Nadir and Benjamin Brenner
S E CTI O N D: T H E RAPEUTIC AGE NTS
1 04. Overview of Therapeutic Agents Used
in Th rombotic Disorders 1 2 53
Sam Schulman and Victor J. Marder
1 0 5. Pharmacology and Mode of Action of Heparin
and G lycosam i noglycans . 1 2 58
Meyer Michel Samama, Jeanine M. Walenga, Evi Kalodiki,
and Jawed Fareed
1 06. Vitamin K Antagonists: Biochemistry,
Pharmacology, and Management 1 26 7
Nirmish Shah, Thomas L. Orte!, and Sam Schulman
1 0 7. Hemorrhagic Complications
of Anticoagulation . 1 2 8 1
Sam Schulman and Mark A . Crowther
1 08. Hepari n – I nduced Thrombocytopenia . 1 292
Theodore E. Warkentin and Andreas Greinacher
xxiv CONTENTS
1 09. Coumari n – l nduced Skin Necrosis and Venous
Limb Gangrene 1 308
Theodore E. Warkentin
1 1 0. New Antithrombotic Drugs . 1 3 1 8
Jeffrey I . Weitz and Peter L. Gross
1 1 1 . Anti platelet Therapy . 1 33 7
Carlo Patrono
1 1 2 . Fou ndations of Thrombolytic Therapy 1 349
Victor J. Marder and Daphne B. Stewart
1 1 3. Anticoagulant and Procoagulant Protei ns
in the Animal Ki ngdom . 1 36 1
Angelina Wei Ling Tan, Shiyang Kwong, and R. Manjunatha Kini
1 1 4. Mechanical Devices and Embolectomy . 1 3 70
Herve Decousus, Patrick Mismetti, and Nicolas Meneveau
PART V:
HEMOSTATIC AND TH ROMBOTIC
DISORDERS ASSOCIATED WITH
SYSTEM I C CON D ITIONS
1 1 5. Overview of Special Clinical G roups . 1 3 79
Victor J. Marder and Joel S. Bennett
1 1 6. Venous Thromboembolism d u ri ng Pregnancy
and with Hormonal Contraceptives 1 380
Saskia Middeldorp, Mathilde Nijkeuter, and Michie! Coppens
1 1 7. Gestational Vascular Complications 1 393
Benjamin Brenner, Anat Aharon, and Jean-Christophe Gris
1 1 8. Maternal Hemostatic Disorders and Obstetric
Hemorrhage 1 402
Philippe De Moerloose, Franoise Boehlen, and Jean-Christophe Gris
1 1 9. Developmental Hemostasis . 1 4 1 1
Paul Monagle, Vera Ignjatovic, and Leslie Roy Berry
1 20. Bleeding in Neonates and C h i ldren 1 424
Anthony K. C. Chan, Elizabeth A. Chalmers, Sam Schulman,
and Alan D. Michelson
1 2 1 . Venous and Arterial Thromboembolism
i n Children 1 434
Paul Monagle, M. Patricia Massicotte, and Anthony K. Chan
1 2 2. Thrombotic Complications of Sickle
Cell Disease . 1 445
Kenneth I. Ataga and Elliott P. Vichinsky
1 2 3. Th rombohemorrhagic Complications
of Sepsis . 1 454
Marcel Levi
1 24. Hemorrhagic Fevers: Epidemiology,
Pathophysiology, and Clin ical Features . 1 463
L. Gayani Tillekeratne, Jennifer Cohn, and Hillary Dunlevy
1 2 5. Cancer- Related Throm bosis . 1 468
Anna Falanga, Agnes Y. Y. Lee, and Frederick R. Rickles
1 26. Hemostatic Cha llenges in Liver Disease 1 48 1
Charles S . Eby and Stephen H. Caldwell
1 2 7. Hemostatic Abnormalities in Renal D isease . 1 49 1
Miriam Galbusera, Giuseppe Remuzzi, and Patrizia Ondei
1 28. M u ltitrauma Derangements in Hemostasis
and Thrombosis . 1 498
Diane A. Schwartz, Lucy Z. Kornblith, John B. Holcomb,
and Mitchell Jay Cohen
1 2 9. Blood Management in the Cardiovascular
Surgical Patient . 1 504
Lawrence Tim Goodnough and L. Henry Edmunds, Jr.
1 30. Th rombohemorrhagic Events in the Elderly:
Special Considerations . 1 5 1 5
Isabelle Mahe and Ludovic Dronet
Index 1 523
Hemostasis and Thrombosis Basic Principles and Clinical Practice 6th Edition PDF Ebook
About the Authors
Victor J. Marder, MD
Professor of Medicine
David Geffen School of Medicine
at University of California, Los Angeles
Los Angeles, California
Professor of Medicine
David Geffen School of Medicine
at University of California, Los Angeles
Los Angeles, California
William C. Aird, MD
Professor of Medicine
Harvard Medical School
Beth Israel Deaconess Medical Center
Boston, Massachusetts
Joel S. Bennett, MD
Professor of Medicine
Perelman School of Medicine
University of Pennsylvania
Philadelphia, Pennsylvania
Sam Schulman, MD
Professor of Medicine
McMaster University
Hamilton, Ontario, Canada
Gilbert C. White, II, MD
Executive Vice President for Research
BloodCenter of Wisconsin
Milwaukee, Wisconsin
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