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Consultative Hemostasis and Thrombosis E-Book

Consultative Hemostasis and Thrombosis E-Book

Craig S. Kitchens | Barbara A Konkle | Craig M. Kessler

(2018)

Additional Information

Book Details

Abstract

With authoritative coverage of rare and common hemostatic disorders, Consultative Hemostasis and Thrombosis, 4th Edition, keeps you both up to date with all that’s new in this fast-moving field as well as reviewing background and development and citing pertinent classical literature. Broad differential diagnoses are provided, underscoring the editors’ position that correct treatment begins with correct diagnosis. This trusted resource by Drs. Craig S. Kitchens, Craig M. Kessler, Barbara A. Konkle, Michael B. Streiff, and David A. Garcia is designed for rapid reference and critical decision making at the point of care.


Table of Contents

Section Title Page Action Price
Front Cover cover
Inside Front Cover ifc1
Consultative Hemostasis and Thrombosis i
Copyright Page iv
Dedication v
Contributors vii
Preface xi
Table Of Contents xiii
Second half title page xv
I General Information 1
1 The Consultative Process 2
Abstract 2.e1
Keywords 2.e1
Extent of the Consultation 3
Confirmatory Consultation 3
Brief Consultation 4
Comprehensive Consultation 4
Urgent Consultation on a Catastrophically Ill Patient 4
“Undiagnosing” Consultation 4
Telemedicine Consultations 6
Curbside Consultation 6
Reason for Consultation 7
Helping Another Physician 7
Second Opinion Requested by the Primary Physician 7
Second Opinion Requested by the Patient 7
Second Opinion Sought by a Third-Party Payor 8
Other Third Parties 8
Disgruntled Patient or Family 8
Inappropriate Consultations 8
Consultant’s Point of View 9
Duties of the Referring Physician and the Consultant 9
Timing 10
How to Do the Consultation 11
Role of the Clinical Laboratory 11
Recommendations 11
Concerns 12
Outcomes 12
Total Agreement 12
Supporting Consultation 12
Finding Another Physician for the Patient 13
Consultant Assumes Primary Care of the Patient 13
Serious Troubles 13
Redirecting the Thrust of a Workup 13
Major Disagreements Between Physicians 13
Duration of Consultation 14
Noncompliant Patients 14
End-of-Life Issues 14
Family Members 15
When a Diagnosis Is Not Forthcoming 15
When Should a Consultant Request Consultation? 15
References 16
2 A Systematic Approach to the Bleeding Patient 17
Abstract 17.e1
Key Words 17.e1
Introduction 17
Clinical Evaluation 17
Obtaining a Detailed History 18
Have You Ever Experienced a Serious Hemorrhagic Complication During or After a Surgical Procedure? 18
Have You Ever Experienced Excessive Vaginal Bleeding During Pregnancy or Immediately After Childbirth or Perineal Bleeding From an Episiotomy? 19
Have You Experienced Persistent Menorrhagia in the Absence of Fibroids or Other Uterine Abnormalities? 19
Do You Experience Brisk or Prolonged Bleeding After Epistaxis or Minor Cuts or Exaggerated Bruising After Minor Trauma? 20
Have You Ever Developed Hemarthrosis, Retroperitoneal Hematoma, or Soft Tissue Hematoma in the Absence of Major Trauma? 20
Have You Ever Experienced Spontaneous Bleeding, Poor Wound Healing, or Dehiscence of a Surgical Wound? 20
Has Any Member of Your Family Experienced Severe Bleeding Complications, Perhaps Requiring Transfusion of Packed Red Blood Cells? 20
Do You Have Any Known Medical Problems? 21
Do You Take Any Prescription Medications, Over-the-Counter Medications, or Homeopathic Remedies on a Regular Basis? 21
Have You Noticed Any Unusual Rashes or Easy Bruisability? 22
Objective Findings on the Physical Examination 22
Integrating Patient History and Physical Examination Findings With Laboratory Results 23
Basic Laboratory Evaluation of Coagulation and Hemostasis 23
Basic Laboratory Tests to Distinguish Between Platelet and Coagulation Defects 25
Laboratory Assessment of the Procoagulant System 29
Laboratory Monitoring of the Direct Oral Specific Anti–Factor Iia and Anti–Factor Xa Anticoagulants 30
Tests for Lupus Anticoagulants 31
Global Hemostatic Assessment Instruments 32
Formulating Treatment Strategies for Managing Acute Hemorrhagic Episodes: How to Use Coagulation Laboratory Data 33
References 35
II Hemorrhagic Processes 38
3 Hemophilia A and Hemophilia B 39
Abstract 39.e1
Keywords 39.e1
Epidemiology and Genetics 39
Carrier Testing 40
Prenatal Diagnosis 40
Postnatal Diagnosis 40
Clinical Features of the Hemophilias 41
Manifestations Early in Life 41
Intraarticular Bleeding: Hemarthroses and Hemophilic Arthropathy 41
Intramuscular Hemorrhage 42
Hematuria/Hemospermia 42
Intracranial Hemorrhage 43
Gastrointestinal and Oropharyngeal Bleeding 43
Pseudotumor Formation in Hemophilia 44
Laboratory Characteristics 44
Therapeutic Modalities for the Hemophilias 45
Hemophilia Treatment Centers 45
Clotting Factor Replacement Therapy With Coagulation Factor Concentrates 45
Desmopressin 51
Ancillary Treatments 51
Antifibrinolytic Agents 51
Fibrin Glues or Sealants and Hemostatic Preparations 52
Dental Care 52
The Aging Patient 52
Treatment Complications 52
Inhibitors 52
Infectious Complications of Replacement Therapy in Hemophilia 53
Gene Therapy 54
References 55
4 Less Common Congenital Disorders of Hemostasis 59
Abstract 59.e1
Keywords 59.e1
Disorders of Fibrinogen 59
Afibrinogenemia/Hypofibrinogenemia 59
Pathogenesis and Genetics 59
Clinical Manifestations 59
Diagnosis 61
Differential Diagnosis 61
Treatment 61
Dysfibrinogenemia 62
Pathogenesis and Genetics 62
Clinical Manifestations 62
Diagnosis 62
Differential Diagnosis 63
Treatment 63
Prothrombin Deficiency (Hypoprothrombinemia and Dysprothrombinemia) 63
Pathogenesis and Genetics 63
Clinical Manifestations 64
Diagnosis 64
Differential Diagnosis 64
Treatment 64
Factor V Deficiency 65
Pathogenesis and Genetics 65
Clinical Manifestations 65
Diagnosis 65
Differential Diagnosis 65
Treatment 66
Factor VII Deficiency 66
Pathogenesis and Genetics 66
Clinical Manifestations 66
Diagnosis 67
Differential Diagnosis 67
Treatment 67
Factor X Deficiency 67
Pathogenesis and Genetics 67
Clinical Manifestations 67
Diagnosis 68
Differential Diagnosis 68
Treatment 68
Factor XI Deficiency 68
Pathogenesis and Genetics 69
Clinical Manifestations 69
Diagnosis 69
Differential Diagnosis 69
Treatment 69
Deficiency of Contact Factors 70
Factor XII Deficiency 70
Pathogenesis and genetics. 70
Clinical manifestations. 70
Diagnosis. 70
Differential diagnosis. 70
Treatment. 70
Prekallikrein Deficiency 70
High-Molecular-Weight Kininogen Deficiency 70
Factor XIII Deficiency 71
Pathogenesis and genetics. 71
Clinical manifestations. 71
Diagnosis. 71
Differential diagnosis. 71
Treatment. 71
Familial Combined Factor Deficiencies 71
Combined Factor V/Factor VIII Deficiency (Type I) 72
III Thrombotic Processes 241
14 Thrombophilia 242
Abstract 242.e1
Keywords 242.e1
Introduction 242
Should All Patients With Unprovoked Venous Thromboembolism Receive Lifelong Anticoagulation? 242
Weighing Bleeding Risk 244
Pursuit of Thrombophilia Testing May Divert Attention From More Important Clinical Risk Factors 244
What Are the Pitfalls of Genetic Testing? 244
When Is It Helpful to Test Unaffected Family Members for Genetic Mutations? 245
Pregnancy 245
Estrogen Use 245
Major Surgery 246
Airplane Flights 247
Summary 247
Testing Considerations for Particular Disorders 247
Antithrombin III Deficiency 247
Background 247
Testing 248
Acquired Antithrombin III Deficiencies 248
Clinical Manifestations 249
Protein C Deficiency 249
Background 249
Testing 249
Acquired Protein C Deficiency 249
IV Therapeutic Agents 528
27 Parenteral Antithrombotic Agents 529
Abstract 529.e1
Keywords 529.e1
Introduction 529
Parenteral Antithrombotic Agents 529
Heparin 529
Mechanism and Pharmacology 529
Administration and Monitoring 529
Heparin Resistance 531
Indications and Clinical Use 531
Adverse Effects 531
Reversal of Effect 532
Low-Molecular-Weight Heparin 532
Mechanism and Pharmacology 532
Administration and Monitoring 533
Indications and Clinical Use 533
Adverse Effects 534
Reversal of Effect 534
Pentasaccharides 534
Mechanism and Pharmacology 534
Administration and Monitoring 534
Indications and Clinical Use 534
V Issues Specific to Women 636
31 Thrombotic Risk of Contraceptives and Other Hormonal Therapies 637
Abstract 637.e1
Keywords 637.e1
Basic Science 637
Hormonal Contraceptive Use and Thrombosis 638
Venous Thromboembolism 638
Myocardial Infarction 639
Stroke 639
Hormonal Contraception and Thrombophilia 640
Counseling Thrombophilic Women in Hormonal Contraception Use 640
Hormone Replacement Therapy and Thrombosis 642
Hormone Replacement Therapy and Cardiovascular Disease 642
Hormone Replacement Therapy and Stroke 643
Hormone Replacement Therapy and Venous Thromboembolic Disease 643
Selective Estrogen Receptor Modulators, Aromatase Inhibitors, and Thrombosis 645
Aromatase Inhibitors 645
Estrogen Therapy in Transgender Females 646
Summary 646
References 646
32 Bleeding and the Management of Hemorrhagic Disorders in Pregnancy 651
Abstract 651.e1
Keywords 651.e1
Introduction 651
Normal Placentation 651
Placental Separation and Expulsion 651
Involution of the Uterus 651
Obstetric Bleeding 651
Miscarriage 652
Ectopic Pregnancy 652
Bleeding After the First Trimester of Pregnancy 652
Postpartum Hemorrhage 653
Risk Factors for Postpartum Hemorrhage 653
Preexisting Anemia 653
Thrombocytopenia 653
Obstetric Management of Postpartum Hemorrhage 656
Hematologic Management of Massive Postpartum Hemorrhage 656
Other Hemostatic Agents 657
Laboratory Testing in Management of Postpartum Hemorrhage 658
Pregnancy and Childbirth in Women With Bleeding Disorders 658
Management of Pregnancy and Childbirth in Women With Bleeding Disorders 659
Summary 660
References 660
33 Thrombophilia in Pregnancy 665
Abstract 665.e1
Key Words 665.e1
Introduction 665
Anticoagulant Therapy During Pregnancy 665
Heparin and Heparinoid Compounds 665
Vitamin K Antagonists 666
Parenteral Direct Thrombin Inhibitors 666
Oral Direct Thrombin and Factor Xa Inhibitors (Direct Oral Anticoagulants [DOACs]) 666
Aspirin 667
Thrombolytic Therapy 667
Acute Venous Thromboembolism During Pregnancy 667
Diagnosis of Venous Thromboembolism During Pregnancy 668
Diagnosis of Deep Vein Thrombosis 668
Diagnosis of Pulmonary Embolism 669
Treatment of Venous Thromboembolism During Pregnancy 672
Prevention of Pregnancy-Associated Venous Thromboembolism 673
Prevention of Venous Thromboembolism in Pregnant Women With Thrombophilia and No History of Venous Thrombosis 674
Prevention of Venous Thromboembolism in Pregnant Women With Prior Deep Vein Thrombosis or Pulmonary Embolism 675
Prevention of Venous Thromboembolism During the Postpartum Period 676
Prevention of Placenta-Mediated Pregnancy Complications 678
Thrombophilia and Placenta-Mediated Pregnancy Complications 678
Placenta-Mediated Pregnancy Complications in Women With Antiphospholipid Syndrome 678
Placenta-Mediated Pregnancy Complications in Women Without Antiphospholipid Antibodies or Known Inherited Thrombophilias 680
Placenta-Mediated Pregnancy Complications in Women With Inherited Thrombophilias 682
Peripartum Anticoagulant Management 683
Screening for Thrombophilia 684
References 684
VI Special Issues 695
34 Surgery and Hemostasis 696
Abstract 696.e1
Keywords 696.e1
Surgery for Patients With Congenital Hemostatic Defects 696
Effects of Surgery on Hemostasis 699
Thrombotic Signals as a Result of Surgery 699
Prophylaxis Against Thrombosis 701
Cardiopulmonary Bypass Surgery 702
Orthotopic Liver Transplantation 705
Preoperative Hemostatic Testing 705
Screening for Hemostatic Defects 705
Screening for Thrombotic Disorders 707
Invasive Procedures in Patients With Abnormal Coagulation Tests 708
Massive Transfusion Protocols in Trauma 709
Consultation on Patients With Intraoperative or Postoperative Hemorrhage 709
Closing Comments and Opinions 712
References 713
35 Anticoagulation in the Perioperative Period 721
Abstract 721.e1
Key Words 721.e1
General Approach 721
Thrombosis Risk by Reason for Antithrombotic Therapy 721
Venous Disease 721
Arterial Disease 721
Myocardial Infarction and Stroke 721
Atrial Fibrillation 721
Mechanical Prosthetic Valves 722
Bleeding Risk 723
Recommended Approaches Listed by Antithrombotic Agents 723
Antiplatelet Agents 723
Aspirin 723
P2Y12 Inhibitors 723
Vorapaxar 723
Anticoagulants 724
Heparins 724
Warfarin 724
Continuing Warfarin 724
Bridging 724
Simple Warfarin Interruption 725
Direct Oral Anticoagulants 725
Special Situations 726
Common Procedures 726
Gastrointestinal Procedures 726
Cardiac Device Surgery 726
Percutaneous Coronary Interventions 726
Neuraxial Anesthesia 727
Drug Eluting Stents 727
Antithrombotic Reversal for Emergency Surgery 728
Antiplatelet Agents 728
Warfarin 729
Heparin 729
Direct Oral Anticoagulants 730
Summary 730
References 730
36 Understanding and Managing the Coagulopathy of Liver Disease 734
Abstract 734.e1
Keywords 734.e1
Introduction 734
Hemostatic Alterations in Different Types of Liver Disease 734
Acute Liver Failure 734
Cholestatic Liver Disease 734
Nonalcoholic Fatty Liver Disease 735
Difficulty in Interpreting Hemostasis Test Results in Patients With Liver Disease 735
(Mis)Use of the International Normalized Ratio in Liver Disease 736
The Concept of Rebalanced Hemostasis in Liver Disease 736
Prevention and Treatment of Bleeding Complications (Boxes 36.1 and 36.2) 737
Prophylactic Preprocedural Correction of a Prolonged International Normalized Ratio Is Not Indicated 737
Prophylactic Preprocedural Correction of Moderate Thrombocytopenia Is Not Indicated 738
How to Change Clinical Practice? 739
Wait-and-See: A Rational Approach to Procedural Hemostasis Management 739
Treatment of Spontaneous Bleeding 739
Variceal Bleeding 740
Role of Infection and Renal Failure 740
Thrombotic Complications and Treatment (Boxes 36.3 & 36.4) 740
Venous Thrombosis 740
Arterial Thrombosis 741
Portal Vein Thrombosis 741
Hepatic Artery Thrombosis Following Liver Transplantation 741
Hemostatic Management During Liver Transplantation 741
Conclusion 742
References 742
37 Outpatient Oral Anticoagulant Therapy 747
Abstract 747.e1
Key Words 747.e1
Vitamin K Antagonists 747
Mechanism of Action 747
Pharmacokinetics and Pharmacodynamics 747
Warfarin and Drug Interactions 748
Therapeutic Range and Monitoring 749
Practical Aspects of Warfarin Management 750
Initiation and Maintenance Dosing 750
Management of Nontherapeutic International Normalized Ratios 751
Management of Oral Anticoagulation During Invasive Procedures 752
Diagnostic Evaluation of Bleeding 753
Predicting and Managing the Risks of Oral Anticoagulant Therapy 754
Anticoagulation Management Services 754
Patient Self-Testing and Patient Self-Management 755
Target-Specific, Direct Oral Anticoagulants 756
Limitations of Warfarin 756
Direct Factor IIa Inhibitors 756
Dabigatran 756
Direct Factor Xa Inhibitors 761
Rivaroxaban 761
Apixaban 761
Edoxaban 763
Betrixaban 763
Practical Management of the Direct Oral Anticoagulants 764
Selecting Patients for Direct, Target- or Factor-Specific Oral Anticoagulants Therapy (Atrial Fibrillation and Venous Thromboembolism) 764
Initiation of Direct, Target- or Factor-Specific Oral Anticoagulants Therapy (Atrial Fibrillation and Venous Thromboembolism) 764
Care Transitions and Patient Follow-Up Strategies 766
Monitoring or Measuring Direct, Target- or Factor-Specific Oral Anticoagulants Activity 766
Managing Drug–Drug Interactions 767
Managing Invasive Procedures 768
Managing Bleeding or Urgent Surgery 768
Managing Transitions Between Classes of Anticoagulants 771
Limitations of the Direct Oral Anticoagulants 771
Implications for Clinical Practice 772
References 772
38 Hematologic Interventions for Acute Central Nervous System Disease 778
Abstract 778.e1
Keywords 778.e1
Introduction 778
Acute Ischemic Stroke 778
Antithrombotic Therapy 778
Antiplatelet Agents 778
Anticoagulant Agents 779
Bleeding Risk Associated With Parenteral Anticoagulation 779
Effect in Subgroups 779
Large Vessel Atherosclerotic Stenosis/Occlusion 779
Cardioembolism 779
Hypercoagulable States 780
Thrombolytic Therapy 780
Mechanical Thrombectomy 781
Consultation Strategies 781
Cerebral Venous Thrombosis 782
Consultation Strategies 782
Overview of Central Nervous System Bleeding 782
Spontaneous Intracerebral Hemorrhage 783
Consultation Strategies 783
Treatment 784
Hyperacute Hemostatic Therapy 784
Reversal Strategies With Particular Coagulation Abnormalities 784
Oral Vitamin K Antagonists 784
Oral Direct Thrombin Inhibitors 785
Direct Oral Anticoagulants 785
Oral Antiplatelet Agents 786
Parenteral Anticoagulants 787
Unfractionated Heparin, Low-Molecular-Weight Heparins, and Heparinoids 787
Intravenous Direct Thrombin Inhibitors 787
Thrombolytic Agents 787
GPIIb/IIIa Inhibitors 787
Other Agents With Antithrombotic Effects 788
Systemic Disorders Associated With Bleeding 788
Aneurysmal Subarachnoid Hemorrhage 788
Procoagulant Therapy to Prevent Early Rebleeding 788
Treatment of Vasospasm and Delayed Cerebral Ischemia 788
Traumatic Brain Injury 789
Traumatic Brain Injury–Related Coagulopathy 789
Hematologic Interventions for Traumatic Brain Injury 790
Correction of Coagulation Test Results Before Neurosurgical Procedures 790
Restarting or Initating Antithrombotic Therapy After Central Nervous System Hemorrhage 791
Antithrombotic Therapy in Patients After Intracerebral Hemorrhage 791
Consultation Strategies 793
Antithrombotic Therapy in Patients After Hemorrhagic Conversion of Ischemic Stroke 793
Antithrombotic Therapy in Patients After Subdural Hematoma 793
References 793
39 Paradoxical Embolic Stroke 802
Abstract 802.e1
Keywords 802.e1
Pathophysiology of Patent Foramen Ovale in Stroke 802
Complete Evaluation of A Young Patient With Stroke 802
Medical Management of Patent Foramen Ovale 804
Percutaneous CLOSURE of Patent Foramen Ovale 806
Atrial Septal Defects 807
Atrial Septal Aneurysms 807
Patients With Patent Foramen Ovale and Underlying Hypercoagulability 807
Conclusion 808
References 808
40 Hemorrhage Control and Thrombosis Following Severe Injury 811
Abstract 811.e1
Keywords 811.e1
Introduction 811
Massive Transfusion and the Coagulopathy of Trauma 811
Pathogenesis 811
Clinical Presentation 813
Treatment of Postinjury Coagulopathy 813
Pharmaceutical Treatment of Postinjury Coagulopathy (Recombinant Factor VIIa, Fibrinogen, Prothrombin Complex Concentrates, Tranexamic Acid) 814
Patients With Congenital Bleeding Diatheses 814
Patients With Acquired Bleeding Diatheses and Comorbid Conditions 814
Thrombocytopenia 815
Thrombosis in Trauma Patients 815
Thrombocytosis in Trauma Patients 815
Summary 816
References 816
41 Hemostatic Aspects of Sickle Cell Disease 819
Abstract 819.e1
Keywords 819.e1
Historical Perspective 819
Pathogenesis of Sickle Cell Disease 819
The Red Blood Cell and Hemoglobin S Polymerization 819
Leukocytes 820
Platelets 821
Endothelium 821
Inflammation 822
Hemostatic Changes 822
Pathogenesis 823
Clinical Considerations 824
The Role of Hemostatic Abnormalities in Vaso-Occlusion 824
Thrombophilic Deoxyribonucleic Acid Mutations 826
Red Blood Cell Transfusion 826
Hydroxyurea Therapy 826
Acute Chest Syndrome 827
Etiology and Pathophysiology 827
Clinical Management 828
Pulmonary Hypertension in Sickle Cell Disease 829
Pathophysiology 829
Clinical Management 830
Stroke 831
New Drugs in Sickle Cell Disease 832
References 833
Index 843
A 843
B 846
C 847
D 849
E 850
F 851
G 852
H 852
I 855
J 856
K 856
L 856
M 857
N 857
O 858
P 858
Q 861
R 861
S 862
T 863
U 867
V 867
W 868
X 869
Z 869
Inside Back Cover ibc1