Hemostasis and Thrombosis

Victor J. Marder MD is Professor of Clinical Medicine, Director, Vascular Medicine Program, Los Angeles Orthopaedic Hospital/UCLA; Division of Hematology/Medical Oncology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.

Gilbert C. White II MD is Executive Vice President for Research, Blood Center of Wisconsin, Milwaukee, WI.

Sam Schulman MD ist Professor of Medicine, McMaster University, Hamilton, Ontario, Canada.

Joel S. Bennett MD Professor of Medicine, University of Pennsylvania, Philadelphia, PA

William C. Aird MD is Associate Professor of Medicine, Beth Israel Deaconness Medical Center, Boston, MA.

Part 1: Introduction to the Field of Hemostasis and Thrombosis





Chapter 1. History of the Field

William C. Aird and Gilbert C. White, II



Chapter 2. Molecular Evolution of the Vertebrate Blood Coagulation System

Marianne A. Grant and William C. Aird



Chapter 3. Molecular and Clinical Genetics

Lesley Everett, Karl C. Desch, and David Ginsburg



Chapter 4: Coagulation Enzymology

Sriram Krishnaswamy



Chapter 5: Cell Biology, Protein Processing, and Cell Signaling

Randal J. Kaufman and Laura Popolo



Chapter 6: Cellular and Molecular Immunology

Andrew H. Lichtman



Chapter 7: Biostatistics and Epidemiology: The Basic Science of Clinical Research

Kerry L. Lee



PART II: BASIC HEMOSTASIS AND THROMBOSIS

SECTION A: COAGULATION & FIBRINOLYSIS



Chapter 8: Overview of Basic Coagulation and Fibrinolysis

Gilbert C. White, II, Victor J. Marder, Sam Schulman, William C. Aird, and Joel S. Bennett



Chapter 9: The Blood Coagulation Factors and Inhibitors: Complementary DNAs, Genes and Expression

Dominic W. Chung, Wengfeng Xu, and Earl W. Davie



Chapter 10: Genetics of Coagulation Proteins

Rodney M. Camire and Paris Margaritis



Chapter 11: Tissue Factor and the Initiation and Regulation (TFPI) of Coagulation

George J. Broze, Jr. and James H. Morrissey



Chapter 12: Factors V and VIII

Randal J. Kaufman, Philip J. Fay, Laura Popolo, and Thomas L. Ortel



Chapter 13: Structure and Function of von Willebrand Factor

Sandra L. Haberichter and Robert R. Montgomery



Chapter 14: Structure and Function of Vitamin K-Dependent Coagulant and Anticoagulant Proteins

Kanagasabai Vadivel, Amy E. Schmidt, Victor J. Marder, Sriram Krishnaswamy, and S. Paul Bajaj



Chapter 15A: Factor XI

Peter N. Walsh and David Gailani



Chapter 15B: The Factor XII-driven Plasma Contact System

Thomas Renne



Chapter 16: Fibrinogen Structure and Function

John W. Weisel and Carl-Erik Dempfle

Chapter 17: Factor XIII

Cédric Duval, Helen Philippou, and Robert A. S. Ariëns



Chapter 18: Antithrombin and the Serpin Family

Susan C. Bock



Chapter 19: Protein C and S and Thrombomodulin System

Laurent O. Mosnier and John H. Griffin

Chapter 20: Plasminogen Activation and Regulation of Fibrinolysis

Nuala A. Booth and Nicola J. Mutch



Chapter 21: Inhibitors of Fibrinolysis

Björn Wiman and Anna Ågren



SECTION B: PLATELET BIOLOGY



Chapter 22: Overview of Platelet Structure and Function

Joel S. Bennett and Gilbert C. White, II



Chapter 23: Platelet Production: Cellular and Molecular Regulation

Elisabeth Cramer Bordé and William Vainchenker



Chapter 24: Megakaryocyte Structure and Platelet Biogenesis

Joseph E. Italiano, Jr. and John H. Hartwig



Chapter 25: Thrombopoietin

Kenneth Kaushansky and Ian Hitchcock



Chapter 26A: Major Platelet Glycoproteins: GPIb-IX-V

Michael C. Berndt, and Robert K. Andrews



Chapter 26B: Major Platelet Glycoproteins: GPIIb/IIIa

Feng Ye and Mark H. Ginsberg



Chapter 27: Platelet Glycoprotein Polymorphisms and Relationship to Function and Immunogenicity

Thomas J. Kunicki and Diane J. Nugent



Chapter 28: The Basis for Platelet Adhesion

Brian Savage and Zaverio M. Ruggeri



Chapter 29: Shear Effects on Platelets and the Vessel Wall in the Pathogenesis of Atherothrombosis

David L. Bark Jr., Warwick S. Nesbitt, Angus K.T. Wong, and Shaun P. Jackson



Chapter 30: Platelets and Collagen

Steve P Watson, Richard W Farndale, Masaaki Moroi, and Stephanie M Jung



Chapter 31: Integrin αIIb β3 and Platelet Aggregation

Ana Kasirer-Friede, Kamila Bledzka, Jun Qin, , Edward F. Plow, and Sanford J. Shattil



Chapter 32: Molecular Basis for Platelet Secretion

Shaojing Ye and Sidney W. Whitehart



Chapter 33: Platelet Signal Transduction

Charles S. Abrams and Lawrence F. Brass



Chapter 34: Platelet Prostaglandin Metabolism

Tilo Grosser and Garret A. FitzGerald



Chapter 35: The Role of Platelets in Blood Coagulation

Peter N. Walsh



Chapter 36: Blood-Borne Microparticles

José López and Swaminathan Murugappan



SECTION C: VASCULAR BIOLOGY

William C. Aird



Chapter 37: Overview of Vascular Biology

William C. Aird



Chapter 38: Development of the Vasculature, Angiogenesis, Lymphangiogenesis

Aernout Luttun and Peter Carmeliet



Chapter 39: Rheology and Vessel Wall Stress

Peter F. Davies, Larry V. McIntire, and Juan M. Jiménez



Chapter 40: Nitric Oxide

Bradley A. Maron and Thomas Michel

Chapter 41: Blood Vessel Wall in Health and Disease

Keith R. Brunt, Sonya Hui, Jagdish Butany, and Mansoor Husain



Chapter 42: Endothelial Cell Function

William C. Aird



Chapter 43: Role of Endothelium in Hemostasis

Michael J. Krisinger and Edward M. Conway



Chapter 44: Coagulation-Independent Signaling of the Extrinsic Coagulation Pathway

Wolfram Ruf



Chapter 45: Heparin and Vascular Proteoglycans

Douglas M. Tollefsen and Lijuan Zhang



Chapter 46: Endothelial Cell-Blood Cell Interactions

Klaus Ley



Chapter 47: Leukocyte Function and Contribution to Disease

Edith van de Vijver and Taco W. Kuijpers



Chapter 48: Laboratory Markers of Endothelial Cell Activation and Dysfunction

Eduard Shantsila, Gregory Y.H. Lip, and Robert P. Hebbel



PART III: DISORDERS OF COAGULATION, PLATELETS, AND VESSEL WALL

SECTION A: BLEEDING DISORDERS COAGULATION & FIBRINOLYSIS



Chapter 49: Overview of Inherited Hemorrhagic Disorders

Gilbert C. White, II, Harold R. Roberts, and Victor J. Marder



Chapter 50: Differential Diagnosis of the Bleeding Patient

Jill M. Johnsen and Barbara A. Konkle



Chapter 51: Clinical Manifestations and Therapy of Inherited and Acquired Hemophilia

Edward G.D. Tuddenham



Chapter 52: von Willebrand Disease: Diagnosis, Classification, and Treatment

J. Evan Sadler and David Lillicrap



Chapter 53: Rare Inherited Coagulation Disorders Other than Hemophilia

Pier Mannuccio Manucci, Massimo Franchini, and Flora Peyvandi



Chapter 54: Afibrogenemias and Dysfibrogenemias

Dennis K. Galanakis



Chapter 55: Familial Multiple Coagulation Factor Deficiencies

Bin Zhang and David Ginsburg



Chapter 56: Clinical Disorders of Fibrinolysis

Daphne Stewart, Charles W. Francis, and Victor J. Marder



Chapter 57: Acquired Disorders of Coagulation: The Immune Coagulopathies

Charles RM Hay



Chapter 58: Laboratory Markers of Coagulation and Fibrinolysis

Kenneth A. Bauer and Helen A. Ireland



SECTION B: ACQUIRED & INHERITED PLATELET DISORDERS



Chapter 59: Overview of Acquired and Inherited Clinical Platelet Disorders

Joel S. Bennett



Chapter 60: Differential Diagnosis of Thrombocytopenia

Joel S. Bennett and Charles S. Abrams



Chapter 61: Immune Thrombocytopenic Purpuraand Posttransfusion Purpura

Howard A. Liebman, Douglas B. Cines, and James B. Bussel



Chapter 62: Drug-Induced Thrombocytopenia

Richard H. Aster and James N. George



Chapter 63: Inherited Thrombocytopenias

Paquita Nurden and Alan T. Nurden



Chapter 64: Acquired Nonimmune Thrombocytopenia

Andreas Greinacher and Theodore E. Warkentin



Chapter 65: Hereditary Disorders of Platelet Function

Joel S. Bennett and A. Koneti Rao



Chapter 66: Acquired Disorders of Platelet Function

A. Koneti Rao and Joel S. Bennett



Chapter 67: Laboratory Markers of Platelet Activation

Paul Harrison and Alan D. Michelson



SECTION C: PRIMARY DISORDERS OF BLOOD VESSELS



Chapter 68: Overview of Primary Disorders of Blood Vessels

William C. Aird



Chapter 69: Disorders of Connective Tissue and Extracellulara Matrix

Fransiska Malfait and Anne dePaepeChapter 70: Hereditary Hemorrhagic Telangiectasia

Michelle Letarte and Claire L. Shovlin



Chapter 71: Arteriovenous Malformations and Hemangiomas and Cerebral Cavernous Malformations

Douglas A. Marchuk and Mark L. Kahn



Chapter 72: Aneurysms (Large and Small Arteries)

Carlos E. Sanchez, Sangita Kapur, and Neal L. Weintraub



Chapter 73: Intracranial Hemorrhage

Timolaos Rizos and Thorsten Steiner



Chapter 74: Retinopathy

Jing Chen, , Andreas Stahl, and Lois E.H. Smith



SECTION D: THERAPY OF BLEEDING DISORDERS



Chapter 75: Overview to Therapy of Bleeding Disorders

Gilbert C. White II



Chapter 76: Management of Acute Hemorrhage

Nigel S. Key, Micah J. Mooberry, and Rommer Lu



Chapter 77: Therapy with Antifibrinolytic Agents

Daphne B. Stewart and Victor J. Marder



Chapter 78: Transfusion Medicine

Thomas S. Kickler



Chapter 79: New Approaches for the Therapy of Bleeding Disorders, Including Gene Therapy

Gilbert C. White II and Harold R. Roberts



PART IV: THROMBOTIC DISORDERS



SECTION A: VENOUS THROMBOTIC DISORDERS



Chapter 80. Overview of Venous Thromboembolism

Sam Schulman, Victor J. Marder, and Gilbert C. White II



Chapter 81: Thrombophilia Genetics

Joseph Emmerich, Martine Aiach, and Pierre-Emmanuel Morange



Chapter 82: Epidemiology and Risk Factors of Venous Thromboembolism

John A. Heit



Chapter 83: Clinical Manifestations and Diagnosis of Venous Thromboembolism

Henri Bounameaux and Arnaud Perrier



Chapter 84: Venous Thrombosis in Unusual Sites

Victor J. Marder, Hylton V. Joffe, and Sam Schulman



Chapter 85: Sequelae of Venous Thromboembolic Disease: Postthrombotic Syndrome and Chronic Thromboembolic Pulmonary Hypertension

Vittorio Pengo and Susan R. Kahn



Chapter 86: Prevention of Venous Thromboembolic Disease

Menno V. Huisman, Ajay Kakkar, Pasha, and Patrick Mismetti



Chapter 87: Treatment of Venous Thromboembolic Disease

Sam Schulman, Jeffrey Ginsberg, and James Douketis



SECTION B: ARTERIAL THROMBOTIC DISORDERS



Chapter 88: Overview of Arterial Thrombotic Disorders

Sam Schulman and Victor J. Marder



Chapter 89: Pathophysiology of Atherosclerosis

Peter Libby, Paul M. Ridker, and Kevin Croce



Chapter 90: Acute Coronary Syndromes: Manifestations and Management

John Paul Vavalle, John H. Alexander, and Matthew T. Roe



Chapter 91: Pathophysiology of Thromboembolic Stroke

Emer R. McGrath and Martin J. O'Donnell



Chapter 92: Prevention of Thromboembolic Stroke in Patients with Atrial Fibrillation, Valvular Heart Disease, Cardiomyopathy

Thomas Kawano and Jonathan L. Halperin



Chapter 93: Acute Stroke: General Management, Anticoagulant, and Thrombolytic Therapy

Gregory J. del Zoppo and Jeffrey L. Saver



Chapter 94: Medical and Surgical Treatment of Peripheral Arterial Disease

Phillip Joseph, Saurabh Kalra, and Sonia Anand



Chapter 95: Unusual Sites of Arterial Occlusion

Philip A. Kalra, Jon G. Moss, and Peter Wiedemann



Chapter 96: Pathogenesis and Treatment of Biomaterial-Associated Thrombosis

Maud B. Gorbet and Michael V. Sefton



SECTION C: COMPLEX THROMBOTIC DISORDERS



Chapter 97: Overview of Microvascular and Complex Thrombotic Disorders

Theodore E. Warkentine and Victor J. Marder



Chapter 98: Consumptive Thrombohemorrhagic Disorders

Marcel Levi, Donald I. Feinstein, Robert W. Colman, and Victor J. Marder



Chapter 99: Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome-Microangiopathies

George



Chapter 100: Vasculitides

James R. Stone and John H. Stone



Chapter 101: Antiphospholipid Antibodies and the Antiphospholipid Syndrome

Jacob H. Rand and Lucia R. Wolgast



Chapter 102: Thrombohemorrhagic Complications of Myeloproliferative Disorders

Ayalew Tefferi



Chapter 103: Thrombotic and Hemorrhagic Complications Associted with Stem Cell Transplantation

Yona Nadir and Benjamin Brenner



SECTION D: THERAPEUTIC AGENTS



Chapter 104: Overview of Therapeutic Agents used in Thrombotic Disorders

Sam Schulman and Victor J. Marder



Chapter 105: Pharmacology and Mode of Action of Heparin and Glycosaminoglycans

Meyer Michael Samama, Jeanine M. Walenga, Evi Kalodiki, and Jawed Fareed



Chapter 106: Vitamin K Antagonists: Biochemistry, Pharmacology and Management

Nirmish Shah, Thomas L. Ortel, and Sam Schulman



Chapter 107: Hemorrhagic Complications of Anticoagulation

Sam Schulman and Mark A. Crowther



Chapter 108: Heparin-Induced Thrombocytopenia

Theodore E. Warkentin and Andreas Greinacher



Chapter 109: Coumarin-Induced Skin Necrosis and Venous Limb Gangrene

Theodore E. Warkentin



Chapter 110: New Antithrombotic Drugs
Jeffrey I. Weitz and Peter L. Gross



Chapter 111: Antiplatelet Therapy

Carlo Patrono



Chapter 112: Thrombolytic Therapy

Victor J. Marder and Daphne B. Stewart



Chapter 113: Anticoagulant and Procoagulant Proteinsin the Animal Kingdom

Angelina Wei Ling Tan, Shiyang Kwong, and R. Manjunatha Kinia



Chapter 114: Mechanical Devices and Embolectomy

Hervé Decousus, Patrick Mismetti, and Nicolas Meneveau



PART V:Hemostatic and Thrombotic Disorders Associated with Systemic Conditions



Chapter 115: Overview of Special Clinical Groups

Victor J. Marder



Chapter 116: VTE during Pregnancy and with Hormonal Contraceptives

Saskia Middeldorp, Mathilde Nijkeuter, and Michiel Coppens



Chapter 117: Gestational Vascular Complications

Benjamin Brenner, Anat Aharon, Jean-Christophe Gris



Chapter 118: Maternal Hemostatic Disorders and Obstetric Hemorrhage

Philippe De Moerloose, Françoise Boehlen, and Jean Christophe Gris



Chapter 119: Developmental Hemostasis

Paul Monagle, Vera Ignatovic, and Leslie Roy Berry



Chapter 120: Bleeding in Neonates and Children

Anthony K. Chan, Elizabeth A. Chalmers, Sam Schulman, and Alan Michelson



Chapter 121: Venous and Arterial Thromboembolism in Children

Paul Monagle, M. Patricia Massicotte, and Anthony K. Chan



Chapter 122: Thrombotic Complications of Sickle Cell Disease

Kenneth I. Ataga and Elliott P. Vichinsky



Chapter 123: Thrombohemorrhagic Complications of Sepsis

Marcel Levi



Chapter 124: Hemorrhagic Fevers: Epidemiology, Pathophysiology, and Clinical Features

Gayani Tillekeratne, Jennifer Cohn, and Hillary Dunlevy



Chapter 125: Cancer-related Thrombosis

Anna Falanga, Agnes Y. Y. Lee, Frederick R. Rickles



Chapter 126: Hemostatic Abnormalities in Liver Disease

Charles S. Eby and Stephen H. Caldwell



Chapter 127: Hemostatic Abnormalities in Renal Disease

Miriam Galbusera, Giuseppe Remuzzi, and Patrizia Ondei



Chapter 128: Multtrauma Derangements in Hemostasis and Thrombosis

Diane A. Schwartz, Lucy Z. Kornblith, John B. Holcomb, and Mitchell Jay Cohen



Chapter 129: Blood Management in the Cardiovascular Surgery Patient

Lawrence Tim Goodnough and L. Henry Edmunds Jr.



Chapter 130: Thrombo-Hemorrhagic Events in the Elderly

Isabelle Mahe and Ludovic Drouet