Nonmalignant
Hematology
Expert Clinical Review:
Questions and Answers
Syed A. Abutalib
Jean M. Connors
Margaret V. Ragni
Editors
123
,Contents
Red Blood Cells
Evaluation of Anemia in Children and Adults . . . . . . . . . . . . . . . . . . . . 3
Peter W. Marks
Iron Homeostasis and the Pathophysiology and
Management of Iron Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Gordon D. McLaren, Roman L. Kleynberg, and Gregory J. Anderson
Porphyrias: Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . 23
Peter V. Tishler
Disorders of Hemoglobin Synthesis: Pathophysiology
and Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Elena Cassinerio, Giovanna Graziadei, and Maria Domenica Cappellini
Management of Thalassemias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Sherif M. Badawy and Alexis A. Thompson
Allogeneic Hematopoietic Cell Transplant
in β-Thalassemia Major . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
Syed A. Abutalib
Sickle Cell Disease: Prevention of Complications . . . . . . . . . . . . . . . . 63
Enrico Maria Novelli
Sickle Cell Disease: Management of Complications . . . . . . . . . . . . . . 75
Michael Winstead and Elliott Vichinsky
Allogeneic Hematopoietic Cell Transplant
in Sickle Cell Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Santosh L. Saraf
Anemia of Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97
Robert T. Means Jr.
Iron Overload: Diagnosis, Complications, and Management . . . . . . 103
Pierre Brissot
Megaloblastic and Nutritional Anemias . . . . . . . . . . . . . . . . . . . . . . . 113
Sally P. Stabler
ix
,x Contents
Sideroblastic Anemias: Diagnosis and Management . . . . . . . . . . . . . 125
Eric J. Werner and Anthony D. Villella
Primary Autoimmune Warm Antibody Hemolytic Anemias . . . . . . 137
Maria Theresa Krauth and Klaus Lechner
Intrinsic Hemolytic Anemias: Pathophysiology, Diagnosis,
and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153
Charles T. Quinn
Platelets and Coagulation System
Platelet Disorders: Diagnostic Tests and Their Interpretations . . . . 171
Scott F. Huntington, Mark H. O’Hara, and Joel S. Bennett
Inherited Platelet Disorders: Diagnosis and Management . . . . . . . . 185
Natthapol Songdej and A. Koneti Rao
Acquired Platelet Disorders: Diagnosis and Management . . . . . . . . 199
Cindy Neunert
Immune-Mediated Thrombocytopenia . . . . . . . . . . . . . . . . . . . . . . . . 209
Nikolaos Papadantonakis and Keith R. McCrae
Coagulation Cascade and Fibrinolysis Pathway:
Assessment in the Laboratory . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
Lindsey A. George and Michele P. Lambert
Abnormalities in the Fibrinolysis Pathway
and Clinical Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235
Hau C. Kwaan and Brandon J. McMahon
Congenital Disorders of Fibrinogen: Clinical Presentations,
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243
Alessandro Casini and Philippe de Moerloose
Hemophilia A and B: Diagnosis and Management . . . . . . . . . . . . . . 255
Deborah Brown
Coagulation Factor Inhibitors: Diagnosis and Management . . . . . . 263
Birgit M. Reipert and C.L. Kempton
Rare Coagulation Factor Deficiencies:
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273
David Green and Axel Matzdorff
von Willebrand Disease: Differential Diagnosis and Diagnostic
Approach to Specific Subtypes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285
Margaret V. Ragni
von Willebrand Disease: Prevention of Complications
and Management of the Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295
Peter A. Kouides
Antifibrinolytics: Indications and Precautions . . . . . . . . . . . . . . . . . 313
Munjid Al Harthy and Peter Kouides
, Contents xi
Gene Therapy for Bleeding Disorders . . . . . . . . . . . . . . . . . . . . . . . . 321
Paul E. Monahan and Yasmina L. Abajas
Coagulopathy in Systemic Diseases
Disseminated Intravascular Coagulation . . . . . . . . . . . . . . . . . . . . . . 339
Molly W. Mandernach and Craig S. Kitchens
Coagulopathy in Critically Ill Subjects . . . . . . . . . . . . . . . . . . . . . . . . 349
Marcel Levi
Trauma-Associated Coagulopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . 361
John R. Hess
Coagulation-Related Issues in Malignant Hematology:
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
Jason N. Barreto and Mrinal M. Patnaik
Unmet Clinical Needs of Antithrombotic Treatment
in BCR/ABL-Negative Myeloproliferative Neoplasms . . . . . . . . . . . 379
Bianca Rocca and Valerio De Stefano
Bleeding and Thrombosis in a Cancer Patient . . . . . . . . . . . . . . . . . . 395
Annemarie E. Fogerty and Jean M. Connors
Management of the Surgical Patient with Thrombotic
and Bleeding Diathesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 403
Rajiv K. Pruthi
Thrombosis and Therapeutics
Prevention and Treatment of Arterial Thromboembolism . . . . . . . . 417
Michael Weinreich and Joe F. Lau
Prevention of Venous Thromboembolism . . . . . . . . . . . . . . . . . . . . . . 429
Christopher Dittus, Shayna Sarosiek, and Jack Ansell
Diagnostic, Prognostic, and Therapeutic Challenges
in Venous Thromboembolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Ilana Kopolovic, Cynthia Wu, and Agnes Y.Y. Lee
Complications of Venous Thromboembolic Disease . . . . . . . . . . . . . 463
Gregory C. Connolly and Peter Kouides
Hereditary Thrombophilias: Pathophysiology, Timing
of Testing and Familial Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475
Jennifer Curnow, Leonardo Pasalic, and Emmanuel J. Favaloro
Antiphospholipid Antibodies and Syndrome:
Complexities in Diagnosis and Management . . . . . . . . . . . . . . . . . . . 485
Karen Schreiber, Savino Sciascia, and Beverley J. Hunt
Unidentifiable Thrombophilia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 495
Simon Mantha and Gerald A. Soff
Hematology
Expert Clinical Review:
Questions and Answers
Syed A. Abutalib
Jean M. Connors
Margaret V. Ragni
Editors
123
,Contents
Red Blood Cells
Evaluation of Anemia in Children and Adults . . . . . . . . . . . . . . . . . . . . 3
Peter W. Marks
Iron Homeostasis and the Pathophysiology and
Management of Iron Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Gordon D. McLaren, Roman L. Kleynberg, and Gregory J. Anderson
Porphyrias: Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . 23
Peter V. Tishler
Disorders of Hemoglobin Synthesis: Pathophysiology
and Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Elena Cassinerio, Giovanna Graziadei, and Maria Domenica Cappellini
Management of Thalassemias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Sherif M. Badawy and Alexis A. Thompson
Allogeneic Hematopoietic Cell Transplant
in β-Thalassemia Major . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
Syed A. Abutalib
Sickle Cell Disease: Prevention of Complications . . . . . . . . . . . . . . . . 63
Enrico Maria Novelli
Sickle Cell Disease: Management of Complications . . . . . . . . . . . . . . 75
Michael Winstead and Elliott Vichinsky
Allogeneic Hematopoietic Cell Transplant
in Sickle Cell Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Santosh L. Saraf
Anemia of Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97
Robert T. Means Jr.
Iron Overload: Diagnosis, Complications, and Management . . . . . . 103
Pierre Brissot
Megaloblastic and Nutritional Anemias . . . . . . . . . . . . . . . . . . . . . . . 113
Sally P. Stabler
ix
,x Contents
Sideroblastic Anemias: Diagnosis and Management . . . . . . . . . . . . . 125
Eric J. Werner and Anthony D. Villella
Primary Autoimmune Warm Antibody Hemolytic Anemias . . . . . . 137
Maria Theresa Krauth and Klaus Lechner
Intrinsic Hemolytic Anemias: Pathophysiology, Diagnosis,
and Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153
Charles T. Quinn
Platelets and Coagulation System
Platelet Disorders: Diagnostic Tests and Their Interpretations . . . . 171
Scott F. Huntington, Mark H. O’Hara, and Joel S. Bennett
Inherited Platelet Disorders: Diagnosis and Management . . . . . . . . 185
Natthapol Songdej and A. Koneti Rao
Acquired Platelet Disorders: Diagnosis and Management . . . . . . . . 199
Cindy Neunert
Immune-Mediated Thrombocytopenia . . . . . . . . . . . . . . . . . . . . . . . . 209
Nikolaos Papadantonakis and Keith R. McCrae
Coagulation Cascade and Fibrinolysis Pathway:
Assessment in the Laboratory . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
Lindsey A. George and Michele P. Lambert
Abnormalities in the Fibrinolysis Pathway
and Clinical Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235
Hau C. Kwaan and Brandon J. McMahon
Congenital Disorders of Fibrinogen: Clinical Presentations,
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243
Alessandro Casini and Philippe de Moerloose
Hemophilia A and B: Diagnosis and Management . . . . . . . . . . . . . . 255
Deborah Brown
Coagulation Factor Inhibitors: Diagnosis and Management . . . . . . 263
Birgit M. Reipert and C.L. Kempton
Rare Coagulation Factor Deficiencies:
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273
David Green and Axel Matzdorff
von Willebrand Disease: Differential Diagnosis and Diagnostic
Approach to Specific Subtypes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285
Margaret V. Ragni
von Willebrand Disease: Prevention of Complications
and Management of the Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295
Peter A. Kouides
Antifibrinolytics: Indications and Precautions . . . . . . . . . . . . . . . . . 313
Munjid Al Harthy and Peter Kouides
, Contents xi
Gene Therapy for Bleeding Disorders . . . . . . . . . . . . . . . . . . . . . . . . 321
Paul E. Monahan and Yasmina L. Abajas
Coagulopathy in Systemic Diseases
Disseminated Intravascular Coagulation . . . . . . . . . . . . . . . . . . . . . . 339
Molly W. Mandernach and Craig S. Kitchens
Coagulopathy in Critically Ill Subjects . . . . . . . . . . . . . . . . . . . . . . . . 349
Marcel Levi
Trauma-Associated Coagulopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . 361
John R. Hess
Coagulation-Related Issues in Malignant Hematology:
Diagnosis and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
Jason N. Barreto and Mrinal M. Patnaik
Unmet Clinical Needs of Antithrombotic Treatment
in BCR/ABL-Negative Myeloproliferative Neoplasms . . . . . . . . . . . 379
Bianca Rocca and Valerio De Stefano
Bleeding and Thrombosis in a Cancer Patient . . . . . . . . . . . . . . . . . . 395
Annemarie E. Fogerty and Jean M. Connors
Management of the Surgical Patient with Thrombotic
and Bleeding Diathesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 403
Rajiv K. Pruthi
Thrombosis and Therapeutics
Prevention and Treatment of Arterial Thromboembolism . . . . . . . . 417
Michael Weinreich and Joe F. Lau
Prevention of Venous Thromboembolism . . . . . . . . . . . . . . . . . . . . . . 429
Christopher Dittus, Shayna Sarosiek, and Jack Ansell
Diagnostic, Prognostic, and Therapeutic Challenges
in Venous Thromboembolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Ilana Kopolovic, Cynthia Wu, and Agnes Y.Y. Lee
Complications of Venous Thromboembolic Disease . . . . . . . . . . . . . 463
Gregory C. Connolly and Peter Kouides
Hereditary Thrombophilias: Pathophysiology, Timing
of Testing and Familial Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475
Jennifer Curnow, Leonardo Pasalic, and Emmanuel J. Favaloro
Antiphospholipid Antibodies and Syndrome:
Complexities in Diagnosis and Management . . . . . . . . . . . . . . . . . . . 485
Karen Schreiber, Savino Sciascia, and Beverley J. Hunt
Unidentifiable Thrombophilia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 495
Simon Mantha and Gerald A. Soff
