ISBN-10:
0316457310
ISBN-13:
9780316457316
Pub. Date:
04/28/1996
Publisher:
Lippincott Williams & Wilkins
Blood: Textbook of Hematology / Edition 2

Blood: Textbook of Hematology / Edition 2

by James H. Jandl

Hardcover

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Product Details

ISBN-13: 9780316457316
Publisher: Lippincott Williams & Wilkins
Publication date: 04/28/1996
Edition description: Second Edition
Pages: 1510
Product dimensions: 9.06(w) x 11.42(h) x (d)

Table of Contents

CONSOLIDATION AND MAINTENANCE THERAPY
877(1)
Consolidation
877(1)
Maintenance
878(1)
Curbing Emesis, the Curse of Chemotherapy
878(1)
Therapy Should Be PDQ
878(1)
IMMUNOTOXINS AND OTHER AGENTS ON TRIAL
878(1)
Antisense Oligonucleotide Therapy Makes Sense
878(1)
Chemical Induction of Differentiation
879(1)
Marrow Transplantation
879(24)
SECURING AND SELECTING DONORS
879(1)
Use of Haplocompatible Marrow
879(1)
MARROW TRANSPLANTATION GIVEN DURING FIRST REMISSION
880(1)
Resurrection: Measures of Engraftment
880(1)
Leukemic Relapse in Donor Cells
881(1)
AUTOLOGOUS MARROW TRANSPLANTATION: PURGING OF LEUKEMIC CELLS
881(1)
AUTOLOGOUS BLOOD STEM CELL TRANSPLANTATION
881(1)
Stem Cell Mobilization
882(1)
ALLOGENEIC STEM CELLS: THE NEW WAVE
882(1)
PREVENTION AND TREATMENT OF GVHD
882(2)
Acute GVHD: A Cytokine Explosion
883(1)
Chronic GVHD: Exemplar of Autoimmunity
884(1)
PREVENTION OF GVHD
884(2)
Cyclosporine Inhibits Lymphokine Production
884(1)
T Cell Purging by Immunotoxins or Selective Depletion: A Flawed Goal
885(1)
Can GVL Effect and GVHD Be Separated?
886(1)
Treatment of Established GVHD
886(1)
OTHER COMPLICATIONS
886(1)
Posttransplantation Immunodeficiency
886(1)
Infectious Complications
886(1)
Virus Infections
887(1)
Interstitial Pneumonitis
887(1)
SWEET ARE THE SUCCESSES
887(16)
22. Chronic Myeloproliferative Syndromes 903(58)
CHRONIC MYELOGENOUS LEUKEMIA
903(20)
Pathophysiology
903(5)
MOLECULAR BIOLOGY OF THE Ph TRANSLOCATION: REPRISE
903(3)
The Philadelphia Story
903(1)
The abl-bcr Connection
904(1)
The bcr-abl Fusion Gene Generates a Hyperactive Tyrosine Kinase
904(1)
Bcr-Abl Oncoproteins Induce Constitutive Phosphorylation and Block Apoptosis
905(1)
Ph-Positive Acute Lymphatic Leukemia Redux
905(1)
Ph-Positive Acute Myelogenous Leukemia
906(1)
Ph-Negative CML
906(1)
Ph Mosaicism
906(1)
ADVANTAGES AND DISADVANTAGES OF CML CELLS
906(1)
CYTOKINETIC AND CELL CULTURE STUDIES OF CML
907(1)
Cell Culture Studies
907(1)
Other Proliferative Advantages of CML Cells
907(1)
Functional and Proliferative Defects in CML Cells
907(1)
CLONAL EVOLUTION: BLAST CRISIS
907(1)
Description
908(2)
CLINICAL FEATURES OF THE CHRONIC PHASE
908(1)
UNUSUAL FEATURES OF CML
908(2)
Secondary Myelofibrosis
909(1)
Leukostasis
909(1)
CNS Involvement
909(1)
Hypercalcemia and Osteolytic Bone Lesions
910(1)
Bleeding and Thrombosis
910(1)
Basophilia with Hyperhistaminemia
910(1)
Hematologic Findings
910(6)
CHRONIC PHASE
911(2)
Leukocyte Counts
911(1)
Anemia
912(1)
Platelet Counts
912(1)
Morphologic Abnormalities of Blood Cells
912(1)
Marrow
912(1)
Leukocyte Alkaline Phosphatase
912(1)
Other Laboratory Abnormalities
913(1)
ACCELERATED PHASE (ACUTE TRANSFORMATION)
913(2)
BLAST CRISIS
913(2)
Hematologic Changes During Myeloid Blast Crisis
915(1)
Most Lymphoid Blast Crises Are of B Cell Origin
915(1)
Rarely Blast Crisis Is of T Cell Origin
915(1)
Small Solace
915(1)
CML IN CHILDREN
915(1)
Juvenile CML
915(1)
Neurofibromatosis and Myeloproliferative Syndromes
916(1)
Course and Prognosis
916(1)
CAUSES OF DEATH
917(1)
Treatment
917(4)
CONVENTIONAL CHEMOTHERAPY FOR CHRONIC PHASE CML
917(3)
Busulfan
917(1)
Busulfan Toxicity
918(1)
Hydroxyurea, A Superior Alternative to Busulfan
918(1)
Intensive Combination Chemotherapy Extends Survival Somewhat
919(1)
INTERFERONS ELICIT CLONAL REMISSIONS
920(1)
IFN alfa Following Combination Chemotherapy
920(1)
OTHER FORMS AND COMBINATIONS OF THERAPY FOR THE CHRONIC PHASE
920(1)
Acute Cytoreduction: Leukapheresis, Splenic Irradiation
920(1)
Total Body Irradiation
921(1)
Immunotherapy
921(1)
MANAGEMENT OF THE ACCELERATED PHASE
921(1)
MANAGEMENT OF BLAST CRISES
921(1)
Myeloid Blast Crisis
921(1)
Lymphoid Blast Crisis
921(1)
Marrow Transplantation
921(2)
Results: Blast Phase Versus Chronic Phase CML
921(1)
SYNGENEIC MARROW TRANSPLANTS
921(1)
ALLOGENEIC MARROW TRANSPLANTATION
922(1)
Graft-Versus-Leukemia
922(1)
A Modicum of GVHD Helps
922(1)
Unrelated Donor Marrow Transplantation for CML
922(1)
Leukemic Transformation of Engrafted Marrow
922(1)
AUTOLOGOUS STEM CELL TRANSPLANTATION
923(1)
IDIOPATHIC MYELOFIBROSIS
923(8)
Pathogenesis and Pathophysiology
924(1)
Regulation of Marrow Collagen
924(1)
DYSMEGAKARYOCYTOPOIESIS, GROWTH FACTORS, AND THE C-SIS ONCOGENE
924(1)
Description
925(2)
SPLENOMEGALY
925(1)
EXTRAMEDULLARY HEMATOPOIESIS
925(2)
Extramedullary Hematopoietic Tumors
927(1)
OSTEOSCLEROSIS AND OTHER SKELETAL DEFECTS
927(1)
Hematologic Findings
927(3)
MORPHOLOGY
927(3)
Effects of Splenectomy on Morphology
928(1)
Anemia
928(1)
White Counts
929(1)
Platelet Levels and Hemostasis
929(1)
Marrow Findings
929(1)
Differential Diagnosis
930(1)
IDIOPATHIC VERSUS SECONDARY MYELOFIBROSIS: URINARY HYDROXYPROLINE
930(1)
TERMINATION OF POLYCYTHEMIA VERA
930(1)
MYELOFIBROSIS VERSUS CML
930(1)
Treatment
930(1)
SPLENECTOMY REDUX
931(1)
MARROW TRANSPLANTATION
931(1)
Prognosis
931(1)
POLYCYTHEMIA VERA
931(9)
Pathophysiology
932(2)
LATE PV PROGENITORS ARE INDEPENDENT OF BOTH EP AND EP RECEPTOR
932(2)
EP Receptor Revisited
932(1)
The Untracking of PV Investigations: A Possible Role for IGF-1
932(1)
Familial Polycythemia Vera
933(1)
Erythremia
933(1)
Chromosomal Abnormalities
933(1)
Extreme Mosaicism Versus Somatic Selection
934(1)
Description
934(1)
Aquagenic Pruritus
934(1)
CIRCULATORY DISTURBANCES OF THE CNS
934(1)
CARDIOVASCULAR AND RESPIRATORY PROBLEMS
934(1)
SPLENOMEGALY
935(1)
PV AND PREGNANCY
935(1)
Hematologic and Laboratory Findings
935(3)
Red Cell Counts
935(1)
Granulocytes
936(1)
Platelets
936(1)
MARROW
936(1)
Quantitative Evaluation of Marrow Composition
937(1)
POSTPOLYCYTHEMIC MYELOFIBROSIS
937(1)
AML IN PV
938(1)
OTHER LABORATORY FINDINGS
938(1)
Uric Acid
938(1)
Cobalamin Binding Protein
938(1)
Differential Diagnosis
938(1)
Treatment
938(2)
PHLEBOTOMY
938(1)
CHLORAMBUCIL
939(1)
(32)P
939(1)
HYDROXYUREA, THE DRUG OF CHOICE
939(1)
TREATMENT OF POSTPOLYCYTHEMIC MYELOFIBROSIS AND LEUKEMIC TRANSFORMATION
939(1)
INTERFERON TO THE RESCUE?
940(1)
ESSENTIAL THROMBOCYTHEMIA
940(21)
Pathophysiology
940(1)
CYTOGENETICS
940(1)
THROMBOKINETICS
940(1)
THROMBOCYTHEMIA AND PV ARE RELATED DISORDERS
941(1)
PLATELET ABNORMALITIES
941(1)
Exclusion of Secondary (Reactive) Thrombocytosis
941(1)
Description
942(1)
BLEEDING
942(1)
THROMBOSIS
942(1)
Disturbance of the Microvasculature: Erythromelagia
942(1)
Hematologic Findings
942(1)
MARROW
942(1)
LEUKEMIC METAMORPHOSIS
943(1)
OTHER PATTERNS OF TRANSFORMATION
943(1)
Management
943(18)
BLEEDING AND THROMBOSIS
943(1)
Antiaggregating Agents in Controlling Thrombotic Complications
944(1)
Splenectomy Is Usually Counterindicated
944(1)
Recombinant Interferon Alfa
944(1)
Anagrelide: Definitive Therapy for Thrombocythemia?
945(16)
23. Acute Lymphatic Leukemia 961(30)
INCIDENCE, CLASSIFICATION, AND PATHOPHYSIOLOGY
961(7)
Genetic Basis for ALL
961(5)
AD HOX FUNCTIONS OF CHIMERIC PROTEINS: AN OVERVIEW
961(1)
IMMUNOLOGIC CLASSIFICATION
962(2)
B Cell Developmental Markers
962(1)
T Cell Developmental Markers
962(1)
ALL with Myeloid-Associated Antigens
963(1)
Monotonous Immune Receptor Gene Rearrangements Are Markers of Clonal Proliferation
963(1)
Detection of Minimal Residual Leukemia
963(1)
HYBRID, MIXED LINEAGE, AND LINEAGE-SWITCHED ACUTE LEUKEMIAS
964(2)
Lineage Switching
964(1)
Biphenotypic ALL
964(2)
Morphologic Classification
966(1)
Influence of Age on Incidence of Morphologic Types
966(1)
Morphologic Versus Immunologic Indicators of Prognosis
966(1)
An Overview of Prognostic Indicators
967(1)
Cytogenetics in ALL: Favorable and Unfavorable Prognostic Indicators
967(1)
t(9;22)
967(1)
t(4;11)
967(1)
t(8;14)
968(1)
OTHER CONSISTENT CHROMOSOME ABNORMALITIES IN ALL
968(1)
6q-
968(1)
t(1;19)(q23;p13.3)
968(1)
(del)9p21-22 and Lymphomatous ALL
968(1)
HYPERDIPLOIDY IS A FAVORABLE PROGNOSTIC INDICATOR
968(1)
DESCRIPTION
968(4)
BONE AND JOINT INVOLVEMENT
969(1)
Extramedullary ALL
969(3)
CNS LEUKEMIA
970(1)
CNS Is the Major Site of Potential Relapse in ALL
970(1)
MEDIASTINAL AND HILAR INVOLVEMENT
970(1)
TESTICULAR INVOLVEMENT
971(1)
MISCELLANEOUS MANIFESTATIONS
972(1)
HEMATOLOGIC FINDINGS
972(4)
Blood Cells
972(3)
WHITE CELLS
972(1)
CYTOCHEMICAL PROFILE
973(2)
PAS Reaction
973(1)
TdT
974(1)
CD10
974(1)
Acid Phosphatase
974(1)
Other Markers
974(1)
Uncommon Morphologic Variants
974(1)
Hand Mirror Cells Give Nonspecific Reflections
974(1)
Inclusion Body ALL (Granular ALL)
975(1)
Hypereosinophilic Syndrome in ALL
975(1)
RED COUNTS AND PLATELET LEVELS
975(1)
Marrow Biopsy Is the Key to Diagnosis
975(1)
OTHER LABORATORY FINDINGS
975(1)
MANAGEMENT
976(65)
Combination Chemotherapy of ALL
976(5)
REMISSION INDUCTION
976(4)
Postinduction Therapy
977(2)
MDR 1 Expression Is Robust in Adults
979(1)
ALL of Infancy
979(1)
Death and Clarification: Quantitating Grief
979(1)
CENTRAL NERVOUS SYSTEM THERAPY
980(1)
Toxicity of CNS Therapy
980(1)
Treatment of Failed CNS Prophylaxis
980(1)
THERAPY OF TESTICULAR RELAPSE
980(1)
TREATMENT OF MARROW RELAPSE
980(1)
Marrow Transplantation
981
ALLOGENEIC MARROW TRANSPLANTATION
981(1)
TREATMENT WITH AUTOLOGOUS MARROW TRANSPLANTS
981(10)
Potboilers
982(9)
24. Chronic Lymphatic Leukemia 991(28)
Cell Biology: CLL Is a Malignancy of CD5(+) B Cells
991(1)
Stings Like a B, Flies Like a T
991(1)
Incidence and Epidemiology
991(1)
CLL Is an Acquired Disorder
991(1)
Age and Sex
991(1)
PATHOPHYSIOLOGY
992(5)
CLL Is a Monoclonal Disease
992(1)
Monoclonality by Light Chain Analysis
992(1)
Clonal Analysis with Anti-idiotypic Antibody
992(1)
B-CLL Cells Are Arrested at Intermediate States of B Cell Maturation
992(4)
IMMUNOPHENOTYPIC SUBCLASSIFICATION
992(1)
B-CLL CELLS USE A LIMITED AND BIASED V GENE REPERTOIRE
992(1)
HYPOGAMMAGLOBULINEMIA AND UNBALANCED IMMUNOGLOBULIN SYNTHESIS
993(1)
Obstipation by Monotypic XXX Chains
993(1)
Biphenotypic B-CLL
993(1)
T CELL FUNCTION IS IMPAIRED IN B-CLL BY INCREASED T SUPPRESSOR CELLS
993(1)
Infection: The Nemesis of CLL Patients
994(1)
CHROMOSOME ABNORMALITIES
994(1)
Interphase Studies
995(1)
13q14 (rb-1) Gene Deletion in B-CLL
995(1)
14q+
996(1)
t(11;14)
996(1)
Cytokinetics
996(1)
DESCRIPTION
997(7)
Systemic Manifestations
997(1)
Local Manifestations
997(2)
SKIN
998(1)
LYMPHADENOPATHY AND SPLENOMEGALY
998(1)
GASTROINTESTINAL TRACT
998(1)
LUNGS
998(1)
NEUROLOGIC COMPLICATIONS
998(1)
Leukoencephalopathy
999(1)
Progressive Multifocal Encephalopathy
999(1)
SKELETAL INVOLVEMENT
999(1)
Hematologic Findings
999(3)
CLL LYMPHOCYTES
999(1)
ANEMIA, THROMBOCYTOPENIA, AND NEUTROPENIA
1000(1)
MARROW FINDINGS
1000(2)
Pure Red Cell Aplasia in B-CLL
1000(1)
Autoimmune Hemolytic Anemia: A Strange Interlude
1001(1)
Some Autoantibodies Are Produced by Clonal B Cells
1001(1)
Detection and Management of AHA
1001(1)
TRANSFORMATION TO ACUTE LEUKEMIA
1002(1)
Richter's Syndrome
1002(2)
DESCRIPTION
1003(1)
Cytology and Histology of Lymph Nodes
1003(1)
Splenic Infiltration by Immunoblasts
1003(1)
Course and Therapy
1003(1)
T CELL CLL AND LEUKEMIAS OF LARGE GRANULAR LYMPHOCYTES
1004(2)
CD4(+) T-CLL
1004(1)
Adult T Cell Leukemia
1004(1)
CD8(+) T-CLL: Large Granular Lymphocyte Leukemia
1004(2)
T-LGL LEUKEMIA
1005(1)
NK-LGL LEUKEMIA
1005(2)
Nonclonal LGL Lymphocytosis
1005(2)
MANAGEMENT OF CLL
1006(13)
Staging and Prognostic Classification of CLL
1007(1)
CLL Is Usually Bearable but Rarely Curable
1007(3)
CORTICOSTEROIDS
1007(1)
Some General Measures
1008(1)
RESPONSE CRITERIA
1008(1)
ALKYLATING AGENTS
1008(1)
Use of Chlorambucil in CLL
1008(1)
COMBINATION CHEMOTHERAPY IN ADVANCED (STAGES B AND C) CLL
1009(1)
PURINE ANALOGS ARE RATIONAL THERAPIES FOR INDOLENT LYMPHOID MALIGNANCIES
1009(1)
Deoxycoformycin
1009(1)
2-Chlorodeoxyadenosine Induces Apoptotic Suicide in Lymphocytes
1010(1)
Fludarabine
1010(1)
Ionizing Radiation
1010(9)
LOCAL IRRADIATION
1010(1)
Splenic Irradiation
1010(1)
Endolymphatic Radiotherapy
1010(1)
TOTAL BODY IRRADIATION
1011(1)
IMMUNOTHERAPY STILL WAITS IN THE WINGS
1011(1)
Budding Therapies
1011(1)
Interferon Alfa
1011(1)
MARROW TRANSPLANTATION
1011(8)
25. Prolymphocytic and Hairy Cell Leukemias 1019(22)
The Big Picture
1019(1)
PROLYMPHOCYTIC LEUKEMIA
1019(5)
B-PLL VERSUS T-PLL
1019(1)
INCIDENCE AND AGE OF ONSET
1019(1)
Pathophysiology
1019(2)
PLL IS NOT A VARIANT OF CLL
1019(1)
T-PLL Phenotype
1020(1)
CYTOGENETICS
1020(1)
Description
1021(1)
Hematologic Findings
1022(1)
MORPHOLOGY OF LEUKEMIC PROLYMPHOCYTES
1022(1)
Routine Light Microscopy
1022(1)
Cytochemical Stains Distinguish B-PLL and T-PLL
1022(1)
Transmission Electron Microscopy (TEM)
1022(1)
Surface Morphology by Scanning Electron Microscopy (SEM)
1022(1)
BLOOD COUNTS AND MARROW FINDINGS
1023(1)
Prolymphocytoid Transformation Is Often the Finale to CLL
1023(1)
Therapy
1023(1)
HAIRY CELL LEUKEMIA
1024(17)
THE HAIRY CELL
1024(1)
Pathophysiology
1024(3)
PHENOTYPE AND ORIGIN OF HAIRY CELLS
1025(1)
Hairy Cells Are Phenotypic Hybrids
1025(1)
HAIRY CELLS ARE A MALIGNANT COUNTERPART OF MARGINAL ZONE B CELLS
1025(1)
CYTOGENETICS
1026(1)
HAIRY CELL VARIANT
1026(1)
Description
1027(2)
THE HAIRY CELL: CLOSER SCRUTINY
1027(1)
Light Microscopy
1027(1)
TEM and SEM
1027(1)
SPLENOMEGALY
1028(1)
LYMPH NODES
1029(1)
PARAPROTEINEMIA
1029(1)
OSTEOLYTIC LESIONS
1029(1)
INVOLVEMENT OF NONHEMATOPOIETIC ORGANS
1029(1)
Cutaneous Involvement
1029(1)
Hematologic Features
1029(2)
BLOOD FINDINGS
1029(1)
Monocyte Depletion
1030(1)
MARROW EXAMINATION
1030(1)
HCL Can Be Prognostically Subclassified by Nuclear Configuration
1030(1)
HCL Presenting as Aplastic Anemia
1031(1)
VASCULITIS
1031(1)
Treatment
1031(3)
Whom to Treat?
1031(1)
Treatment of HCL after Splenectomy
1032(1)
INTERFERON ALFA
1032(1)
2'-DEOXYCOFORMYCIN (PENTOSTATIN)
1032(1)
2-CHLORODEOXYADENOSINE
1033(1)
At Last: A Systemic Cure for HCL
1033(1)
MARROW TRANSPLANTATION
1034(1)
Secondary Complications
1034(1)
INFECTIOUS COMPLICATIONS
1034(1)
SECOND MALIGNANCIES
1035(1)
Splenic Lymphoma with Villous Lymphocytes
1035(6)
Description and Management
1035(6)
26. Multiple Myeloma and Related Plasma Cell Dyscrasias 1041(58)
Incidence and Mortality Rates
1041(1)
PATHOPHYSIOLOGY
1042(20)
Maturation of Malignant B Cells Prepares Them for Residence in Marrow: Role of IL-6
1042(1)
Pathogenesis of MM Reprised
1043(2)
DISSEMINATION OF PRIMITIVE TUMOR CELLS
1043(1)
TUMOR FORMATION IN MARROW
1043(1)
OSTEOCLASTS AND IL-6 CLEAR THE WAY
1043(1)
Myeloma Cells Must Evade the Immunologic Network to Be Productive
1044(1)
ANTI-IDIOTYPIC NETWORK: A STAR WARS DEFENSE
1044(1)
MM PATIENTS ARE DEFICIENT IN HUMORAL BUT NOT CELLULAR IMMUNITY
1044(1)
IgG Catabolism Is Increased in MM
1045(1)
Malignant Plasma Cells Suppress Synthesis of Normal Immunoglobulin
1045(1)
Monoclonal Gammopathy of Undetermined Significance
1045(2)
THE HIGH GLOBAL INCIDENCE OF MGUS IS A TRIBUTE TO THE ANTI-IDIOTYPE NETWORK
1045(1)
FOLLOWUP AND STAGING OF MGUS
1046(1)
Distinction Between MGUS and Malignant Gammopathies Requires Systematic Surveillance
1046(1)
MM MUST OVERTHROW IMMUNITY TO TRIUMPH
1046(1)
Cytokinetics of MM
1047(2)
TUMOR KINETICS AND PROGNOSIS
1047(1)
Stem Cell Assays
1047(1)
Serum XXX(2)-Microglobulin and Plasma Cell Labeling Index
1047(1)
MYELOMA GROWTH IS DRIVEN BY ANTIGEN
1048(1)
Monoclonal Ig Chain Synthesis Is Often Unbalanced or Deranged
1049(1)
Chromosomal Abnormalities
1049(1)
Monoclonal Protein Abnormalities
1049(2)
ELECTROPHORESIS FOR DETECTION
1050(1)
Electrophoresis of Both Serum and Urine
1050(1)
IMMUNOELECTROPHORESIS FOR IDENTIFICATION
1050(1)
Immunofixation and Immunodiffusion as Backups for IEP
1050(1)
Clinical Impact of Monoclonal Paraproteins Correlates with Immunoglobulin Class and Subclass
1051(2)
LIGHT CHAIN DISEASE
1051(1)
Light Chain Disease Is an Aggressive Variant of MM
1051(1)
IgG MYELOMA
1052(1)
IgG3 Is Prone to Cause the Hyperviscosity Syndrome
1052(1)
IgA MYELOMA
1052(1)
IgD MYELOMA
1052(1)
IgE MYELOMA
1052(1)
IgM MYELOMA
1053(1)
BICLONAL GAMMOPATHIES
1053(1)
Class Switching Does Not Disprove Monoclonality
1053(1)
Authentic Biclonality
1053(1)
Biclonal Gammopathy in MM Clinically Resembles Monoclonal IgG Myeloma
1053(1)
Extreme Secretory Imbalance: Variant Myeloma Products
1053(9)
HEAVY CHAIN DISEASES
1053(2)
XXX HCD
1054(1)
XXX HCD
1054(1)
XXX HCD
1055(1)
NONSECRETORY MYELOMA
1055(1)
J Chain Disease: The Extremity of Ig Biosynthetic Failure
1056(1)
Another Weird Variant: Ig Half-Molecules
1056(1)
AMYLOIDOSIS AND MM
1056(3)
Light Chain Amyloid (AL)
1056(1)
Light Chain Deposition Disease (LCDD)
1057(1)
Light and Heavy Chain Deposition Disease (LHCDD)
1057(1)
Heavy Chain Deposition Disease (HCDD)
1057(1)
Amyloid Disease Unrelated to MM
1058(1)
XXX2M Amyloid
1058(1)
Amyloid A (AA): Secondary Amyloidosis
1058(1)
CLINICAL FEATURES OF AL AMYLOIDOSIS
1059(1)
Cardiac Amyloidosis
1059(1)
Miscellaneous Syndromes Caused by AL Amyloid or LCDD
1059(1)
DIAGNOSIS
1060(1)
PROGNOSIS AND TREATMENT
1060(1)
MYELOMA CELLS
1060(2)
Myeloma Cells Are Heterogeneous
1060(1)
A Cytologic Bestiary of Myeloma Cells
1061(1)
Phagocytic Plasma Cells
1061(1)
DESCRIPTION
1062(11)
Skeletal Disease
1062(5)
RADIOLOGIC CHANGES
1062(1)
X-RAY, CT, AND MRI IN MM
1063(1)
MAXILLOFACIAL AND ORBITAL MYELOMA TUMORS
1064(1)
HYPERCALCEMIA
1064(3)
Clinical Effects of Hypercalcemia
1065(1)
Management of Hypercalcemic Complications of Myeloma
1066(1)
Bisphosphonates: Agents of Choice in Chronic Control of Osteolysis
1066(1)
OSTEOSCLEROTIC MYELOMA AND THE POEMS SYNDROME
1067(1)
Hypersusceptibility to Infection
1067(1)
MM IMPAIRS ANTIBODY PRODUCTION AND OTHER B CELL RESPONSES TO INFECTION
1068(1)
Treatment and Prevention
1068(1)
Renal Disease
1068(3)
MYELOMA CAST NEPHROPATHY (BENCE JONES NEPHROSIS)
1068(1)
Acute Renal Failure in Myeloma Cast Nephropathy
1068(1)
Acute Renal Failure in BJ Nephrosis Is Often Reversible
1068(1)
GLOMERULAR LESIONS
1068(1)
Glomerular Lesions Represent Dense Deposit Disease
1069(1)
DISSEMINATED TISSUE DEPOSITION OF PARAPROTEINS
1069(1)
Renal Amyloid Deposits
1069(1)
DIFFUSE XXX CHAIN NEPHROPATHY
1069(1)
TREATMENT OF RENAL FAILURE
1070(1)
Hemodialysis
1070(1)
Renal Transplantation for Endstage Light Chain Nephropathy
1070(1)
Neurologic Malfunctions
1071(1)
SPINAL CORD COMPRESSION
1071(1)
Management of Spinal Cord Compression
1071(1)
INTRACRANIAL MYELOMA; MYELOMATOUS MENINGITIS
1071(1)
SENSORIMOTOR POLYNEUROPATHY
1071(1)
Multifocal Leukoencephalopathy
1072(1)
Hyperviscosity Syndrome
1072(1)
Solitary and Extraosseous Myelomas
1072(1)
SOLITARY OSSEOUS MYELOMAS
1072(1)
Natural Course and Relationship to MM
1072(1)
Therapy
1072(1)
EXTRAOSSEOUS MYELOMA
1073(1)
Natural Course, Therapy, and Relationship to MM
1073(1)
HEMATOLOGIC FINDINGS
1073(2)
Blood
1073(1)
Bone Marrow
1074(1)
MYELOMA CELL MORPHOLOGY
1074(1)
PROGNOSTIC STAGING
1074(1)
Plasma Cell Leukemia
1075(1)
MANAGEMENT
1075(5)
Relief of Pain Is Essential to Maintaining Mobility
1076(1)
Calcitonin Alleviates Skeletal Pain in MM
1076(1)
Stabilization of Skeletal Healing
1076(1)
Chemotherapy of MM
1076(2)
A CLOSER LOOK AT ALKYLATING AGENTS
1077(1)
Melphalan
1077(1)
Cyclophosphamide
1077(1)
MULTIPLE DRUG COMBINATIONS: ESCAPE FROM PLATEAU
1077(1)
TREATMENT OF MM REFRACTORY TO ALKYLATING AGENTS
1078(1)
Irradiation: Second-Line Therapy in Drug-Resistant MM
1078(1)
Interferons in Maintenance Therapy
1078(1)
Marrow Transplantation in Multiple Myeloma
1079(1)
AUTOLOGOUS TRANSPLANTATION
1079(1)
Purge, Schpurge
1079(1)
Declaration of War
1079(1)
MM and Therapy-Related Acute Myelogenous Leukemia
1079(1)
CLINICAL FEATURES OF SECONDARY LEUKEMIA IN MM
1080(1)
Second Neoplasms Other Than AML Are Uncommon in MM
1080(1)
WALDENSTROM'S MACROGLOBULINEMIA
1080(1)
INCIDENCE
1080(1)
Pathophysiology
1080(1)
CYTOGENETICS
1080(1)
Description
1080(2)
MONOCLONAL IgM IN WM OFTEN REACTS WITH DEFINED ANTIGENS
1080(1)
Perseverating Ancestral Genes?
1081(1)
HYPERVISCOSITY SYNDROMES
1081(1)
OCULAR MANIFESTATIONS
1081(1)
NEUROPSYCHIATRIC PROBLEMS
1081(1)
POLYNEUROPATHY OF WALDENSTROM'S MACROGLOBULINEMIA
1081(1)
LYMPHOPLASMACYTIC INFILTRATION OF ORGANS OTHER THAN MARROW AND LYMPH NODES
1082(1)
Pulmonary Lesions
1082(1)
Cutaneous Manifestations
1082(1)
Renal Abnormalities
1082(1)
Hematologic Findings
1082(2)
BLOOD
1082(1)
MARROW
1083(1)
Histologic Staging
1083(1)
OTHER LABORATORY FINDINGS
1083(1)
IgM in WM Interferes with Hemostasis
1084(1)
Treatment
1084(15)
CHEMOTHERAPY
1084(1)
Another Victory for CdA
1085(1)
PLASMAPHERESIS
1085(1)
Mirabile Dictu: Cure by Splenectomy?
1085(14)
27. Hodgkin's Disease 1099(38)
Reed-Sternberg Cells: Enigmatic Variations
1099(1)
Diagnosis of HD
1099(3)
VARIANT RS CELLS ARE ASSOCIATED WITH VARIANT HISTOPATHOLOGIC CLASSES OF HD
1099(1)
Lacunar Cell Variant
1099(1)
Lymphocyte Predominance Variant: Mixed Cell HD
1100(1)
Lymphocyte Depletion
1100(1)
TRACING THE CELL OF ORIGIN: IMMUNOPHENOTYPING
1100(2)
CD30 and CD40 Are Receptor Markers for HD
1101(1)
Cytokines Compound Confusion
1102(1)
PATHOPHYSIOLOGY: HD REPRESENTS A BREAKDOWN IN MACROPHAGE-T CELL COMMUNICATION
1102(1)
CYTOGENETICS
1103(1)
INCIDENCE AND EPIDEMIOLOGY
1103(3)
Age and Prognosis
1103(1)
DEATH FROM "OTHER CAUSES"
1103(1)
IS HD AN INFECTIOUS OR SOCIAL MALIGNANCY?
1104(1)
Climate, Socioeconomic Status, and HD
1104(1)
CLUSTERING, AGGREGATION, AND CONTAGION
1104(1)
Epstein-Barr Virus
1104(1)
Cell-Mediated Immunity Is Impaired in HD
1105(1)
HD T CELLS ARE INHERENTLY UNABLE TO GENERATE ADEQUATE IL-2
1106(1)
HD-AIDS Complicity
1106(1)
Lymphopenia in HD
1106(1)
Immunosuppression May Facilitate but Usually Does Not Initiate Tumor Growth in HD
1106(1)
DESCRIPTION
1106(6)
Adenopathy and Lymphatic Spread
1106(1)
PECULIARITIES OF HODGKIN'S ADENOPATHY
1107(1)
Extranodal Involvement: Hematogenous Migration of RS Cells
1107(1)
Systemic Symptoms of HD
1107(1)
FEVER, SWEATS, AND WEIGHT LOSS
1107(1)
Severe Pruritus Should Be a B Symptom
1107(1)
Important Syndromes and Organ Involvement
1108(4)
MEDIASTINAL HD
1108(1)
Bulky Mediastinal HD
1108(1)
Pulmonary Involvement
1108(1)
Tracheobronchial Compression
1108(1)
SUBDIAPHRAGMATIC HD: COMPARISON OF TECHNIQUES FOR DETECTING (STAGING) ABDOMINAL SPREAD OF HD
1109(1)
Retroperitoneal Lymph Nodes
1109(1)
SPLENIC INVOLVEMENT BY HD
1110(1)
UNCOMMON SITES OF HD
1110(2)
Cutaneous HD
1111(1)
Renal and Gastrointestinal Involvement
1111(1)
Neurologic Involvement: Cord Compression, Lymphomatous Meningitis, and Intracerebral HD
1111(1)
HEMATOLOGIC FINDINGS
1112(1)
Blood
1112(1)
AUTOIMMUNE HEMOLYTIC ANEMIA
1112(1)
THROMBOCYTOPENIA
1112(1)
WHITE COUNTS
1112(1)
Marrow
1112(1)
Nonspecific Histologic Abnormalities in HD Marrow
1113(1)
CLASSIFICATION AND STAGING
1113(6)
Histopathologic Classification of HD
1113(2)
LYMPHOCYTE PREDOMINANCE HD
1113(1)
Nodular LP HD Is a B Cell Lymphoma
1114(1)
NODULAR SCLEROSING HD
1114(1)
NS-Cellular Phase (NSCP)
1114(1)
NS HD Versus Nodular LP HD Complex
1114(1)
MIXED CELLULARITY HD
1115(1)
LYMPHOCYTE DEPLETION HD
1115(1)
Staging
1115(4)
CLINICAL STAGING (CS)
1116(2)
Requiem for the Lymphogram
1118(1)
PATHOLOGIC STAGING (PS)
1118(1)
Risks and Costs of Stagecraft
1119(1)
Distribution of Patients by PS
1119(1)
SUBSTAGING OF HD
1119(1)
SYSTEMATIC RESTAGING
1119(1)
SURVIVAL IN HD IS DETERMINED BY STAGE AND AGE
1119(1)
TREATMENT
1119(6)
Radiation Therapy in HD
1120(2)
RADIATION THERAPY FIELDS
1120(2)
Mantle Field Irradiation
1120(1)
Waldeyer Field Irradiation
1120(1)
Subdiaphragmatic Irradiation
1120(1)
Total Nodal Irradiation
1121(1)
Field Junctions
1121(1)
Block Those Gonads
1121(1)
RADIATION THERAPY OF STAGES IA AND IIA
1122(1)
Early-Stage HD with Bulky Mediastinal Adenopathy
1122(1)
Combined Modality Therapy in Early-Stage HD
1122(2)
Footnote to Success
1122(1)
TREATMENT OF HD IN PREGNANT PATIENTS
1123(1)
STAGE IIIA
1124(1)
ADVANCED HD: STAGES IIIB AND IV
1124(1)
Salvage Chemotherapy: A Springboard to Cure
1124(1)
Autologous Marrow Transplantation in HD
1124(1)
TRIUMPH OVER REFRACTORY HD
1124(1)
Potential Use of Anti-idiotype Vaccines in HD
1125(1)
THE PRICE OF GLORY: COMPLICATIONS OF HD THERAPY REPRISED
1125(12)
RADIATION THERAPY
1125(1)
Combination Chemotherapy
1125(12)
SECOND MALIGNANCIES
1125(1)
Combination Chemotherapy That Includes an Alkylator Causes AML in HD Patients
1125(1)
AN OVERVIEW OF RELATIVE RISKS OF CANCERS IN HD
1126(1)
REPRODUCTIVE, ENDOCRINE, AND PSYCHOSOCIAL MALFUNCTION
1126(1)
INFECTION
1126(11)
28. Non-Hodgkin's Lymphomas 1137(76)
INCIDENCE, EPIDEMIOLOGY, AND ETIOLOGY
1137(4)
AIDS AND THE NHL UPSURGE
1138(1)
Immunosuppression and EBV Reactivation
1138(3)
IMMUNOSUPPRESSIVE DRUGS
1138(1)
EBV AND BURKITT'S LYMPHOMA
1138(2)
Acquired Immunodeficiency and Burkitt's Lymphoma
1139(1)
Endemic Burkitt's Lymphoma Lacks Rearrangements of c-myc
1139(1)
HIV-ASSOCIATED NHL
1140(1)
Pathologic Spectrum of AIDS-Related NHLs
1140(1)
ADULT T CELL LYMPHOMA: REPRISE
1141(1)
Chaos at the Periphery
1141(1)
PATHOGENESIS
1141(7)
Most NHLs Are of Follicle Center Origin
1141(1)
NORMAL FOLLICLE CENTER CELL RESPONSE TO ANTIGEN: B CELL AMPLIFICATION
1141(1)
THE DRAMATIS PERSONAE OF LYMPH NODE FOLLICLES
1142(1)
MURPHY'S LAW AND NHLS
1142(1)
Follicular NHLs Are Less Aggressive Than Diffuse Lymphomas
1142(1)
Chromosome Rearrangements, Oncogenes, and Fragile Sites
1142(5)
t(14;18): THE HALLMARK OF FOLLICULAR NHLS AND OPPONENT OF APOPTOSIS
1145(1)
DETERMINATION OF CLONALITY IN NHLS
1145(2)
Refinements in Measures of Clonality
1145(1)
How to Proceed
1146(1)
Cytokinetic Characteristics
1147(1)
Hyperdiploid Clones Have a Growth Advantage
1147(1)
Mechanisms of Metastasis
1147(1)
CLASSIFICATION OF NHLS: A CATECHISM FOR CLINICIANS
1148(2)
THE AGONIES OF CATALOGING NHLS: A WORKING FORMULATION FOR CLINICAL USAGE
1148(1)
CRITIQUE: THE NEW WF REQUIRES AMENDMENT
1148(2)
The Irony of Lymphoma Grading
1149(1)
WF and the ICD-O
1149(1)
DESCRIPTION
1150(12)
Low-Grade Lymphomas
1150(4)
Watchful Waiting Versus Preemptive Strikes
1150(1)
SMALL LYMPHOCYTIC LYMPHOMA
1151(1)
INTERMEDIATE CELL LYMPHOMA AND LYMPHOPLASMACYTOID VARIANTS
1151(1)
MANTLE CELL LYMPHOMA
1152(1)
T Cell Variant of Small Lymphocytic Lymphomas
1152(1)
Monocytoid B Cell Lymphoma
1152(1)
FOLLICULAR, PREDOMINANTLY SMALL CLEAVED CELL LYMPHOMA
1152(2)
Histologic Conversion
1153(1)
FOLLICULAR MIXED SMALL CLEAVED AND LARGE CELL LYMPHOMA
1154(1)
Intermediate-Grade NHLs
1154(3)
FOLLICULAR PREDOMINANTLY LARGE CELL LYMPHOMA
1154(1)
DIFFUSE SMALL CLEAVED CELL LYMPHOMA
1154(1)
DIFFUSE MIXED SMALL AND LARGE CELL LYMPHOMA
1154(2)
Peripheral T Cell Variant
1155(1)
Lennert's Lymphoma
1156(1)
DIFFUSE LARGE CELL LYMPHOMA
1156(1)
High-Grade NHLs
1157(5)
LARGE CELL IMMUNOBLASTIC LYMPHOMA
1157(2)
B Immunoblastic Lymphoma
1157(1)
T Immunoblastic Lymphoma
1158(1)
Anaplastic Large Cell Lymphoma
1158(1)
LYMPHOBLASTIC LYMPHOMA
1159(1)
T Lymphoblastic Lymphoma Cells Are Phenotypically Heterogeneous
1159(1)
SMALL NONCLEAVED CELL LYMPHOMA
1160(1)
African Burkitt's Versus Sporadic Burkitt's-like Lymphomas
1160(1)
Small Noncleaved Cell Lymphoma: Non-Burkitt's Undifferentiated Variant
1161(1)
LYMPHOMAS OF MACROPHAGES
1161(1)
True Histiocytic Lymphoma
1161(1)
Malignant Histiocytosis
1161(1)
Hemophagocytic Syndromes
1162(1)
T Cell Lymphomas and Hemophagocytic Syndromes
1162(1)
THE NEW KILLER ON THE BLOCK: NK CELL LYMPHOMA
1162(1)
ORGAN INVOLVEMENT IN NHLS
1162(17)
Cutaneous T Cell Lymphoma
1162(6)
CUTANEOUS T CELL LYMPHOMA IS CAUSED BY HTLV INFECTION
1163(1)
MYCOSIS FUNGOIDES AND THE SEZARY SYNDROME
1163(1)
Cytology of Sezary Cells
1163(1)
CTCL Cells Are of Nodal Origin
1164(1)
CUTANEOUS MANIFESTATIONS
1164(1)
EXTRACUTANEOUS MANIFESTATIONS
1165(1)
HISTOPATHOLOGY
1165(1)
STAGING OF CTCL
1166(1)
THERAPY
1166(1)
Systemic Chemotherapy
1166(1)
Cruel Choices in Combating a Cruel Disease
1167(1)
Extracorporeal Photochemotherapy
1167(1)
Radiommunotherapy: Great Pictures but No Cures
1168(1)
NHLs of the Head and Neck
1168(1)
Lymphomas of the Orbit and Adnexa
1168(1)
CNS Involvement
1168(4)
SECONDARY (METASTATIC) NHL
1168(2)
Intracranial Metastases
1169(1)
Spinal Cord Compression
1169(1)
PRIMARY CNS NHL
1170(2)
Therapy of Primary Lymphoma of the CNS
1171(1)
LEPTOMENINGEAL NHL, CSF CYTOLOGY, AND INTRATHECAL THERAPY
1172(1)
Primary Leptomeningeal Lymphoma
1172(1)
INTRATHECAL THERAPY FOR CNS NHL
1172(1)
Gastrointestinal Tract
1172(4)
THE MALT LYMPHOMAS: A LEAGUE OF THEIR OWN
1172(1)
Gut Wrenching Reclassification: The Marginal Zone B Cell Lymphomas
1173(1)
Some MALT Lymphomas Respond to Antibiotics!
1173(1)
PRIMARY GASTRIC LYMPHOMA
1173(2)
Clinical Features and Diagnosis of Gastric Lymphoma
1173(1)
Staging and Management
1174(1)
OTHER PRIMARY GASTROINTESTINAL LYMPHOMAS
1175(1)
IPSID
1175(1)
Management
1175(1)
Other Intestinal NHLs
1175(1)
Heart, Lung, and Great Vessels
1176(1)
SUPERIOR VENA CAVA SYNDROME CAN BE AN ONCOLOGIC EMERGENCY
1176(1)
Other Organs
1177(2)
SPLEEN
1177(1)
LIVER
1177(1)
KIDNEYS
1177(1)
B CELL LYMPHOMAS OF SKIN
1177(2)
NHLS OF THE BREAST
1179(1)
ANGIOTROPIC INTRAVASCULAR LARGE CELL LYMPHOMA
1179(1)
PRELYMPHOMAS AND LYMPHOMATOID DISORDERS
1179(2)
Angiocentric Lymphoma
1180(1)
MANAGEMENT
1180(1)
ANGIOIMMUNOBLASTIC LYMPHADENOPATHY WITH DYSPROTEINEMIA
1180(1)
Lymphomatoid Papulosis
1181(1)
MANAGEMENT
1181(1)
HEMATOLOGIC FINDINGS IN NHLS
1181(3)
Marrow
1182(1)
Biopsy Versus Imaging for Staging NHL
1182(1)
Leukemic Conversion of NHLs
1182(2)
LYMPHOMA CELL LEUKEMIA
1183(1)
Leukemic Conversion in Other NHLs
1184(1)
THERAPY
1184(29)
Staging
1184(3)
CLINICAL STAGING
1184(1)
NONINVASIVE STAGING
1185(1)
CT Scanning of Abdomen and Pelvis
1185(1)
Bipedal Lymphography
1185(1)
Magnetic Resonance Imaging
1186(1)
Radionuclide Scintigraphy
1186(1)
INDICATIONS FOR MORE INVASIVE STAGING
1186(1)
Radiation Therapy
1187(2)
LOW-GRADE NHL: WHEN TO TREAT
1187(1)
Low-Grade NHL Presenting in Symptomatic Stage III or IV
1188(1)
INTERMEDIATE- AND HIGH-GRADE NHLS
1188(1)
Radiotherapy Has Had a Bad Rap
1188(1)
Chemotherapy
1189(3)
COMBINATION CHEMOTHERAPY: THE CHOP FAMILY
1189(3)
The Price
1189(1)
Drugging the Drug Pumps
1189(2)
Chemosensitizers plus Infusional Therapy
1191(1)
Salvage Treatment of Lymphomas
1192(1)
AUTOLOGOUS BLOOD STEM CELL TRANSPLANTATION
1192(1)
MONOCLONAL ANTIBODIES HAVE THE POTENTIAL FOR PURGING AND TARGETING TUMOR CELLS
1193(1)
Targeting T Cell Lymphomas
1193(1)
IMMUNOTOXINS AS SMART MISSILES: THE FIRST SKIRMISHES
1193(1)
Therapy with Monoclonal Anti-idiotype Antibodies
1193(20)
Idiotype-Specific Plasmid-Conveyed Vaccines
1194(19)
29. Hemostasis 1213(64)
HEMOSTATIC RESPONSE TO TRAUMA
1213(1)
VASCULAR AND ENDOTHELIAL FUNCTION IN HEMOSTASIS
1213(11)
The Luminal Aspect of Vascular Endothelium Is Nonthrombogenic
1213(1)
ENDOTHELIAL ADJUSTMENTS TO SHEAR STRESS
1213(1)
Endothelin-1 and Nitric Oxide: Partners in Regulation of Blood Flow
1213(1)
Synthetic and Secretory Functions of Vascular Endothelium
1214(4)
NATURALLY OCCURRING ANTICOAGULANT MECHANISMS: A PREVIEW
1214(1)
PROSTACYCLIN
1215(1)
THROMBOMODULIN AND ACTIVATION OF PROTEIN C
1215(1)
Structure-Function Relationships
1215(1)
Thrombomodulin Structure and Function
1215(1)
Protein S Enhances Binding of Protein C to Phospholipid Surfaces
1216(1)
Protein S Helps Coordinate Coagulation and Complement Function
1216(1)
APC Resistance
1217(1)
ANTITHROMBIN (ANTITHROMBIN III)
1217(1)
Heparin Cofactor II: A Weak Sister Serpin
1218(1)
Platelet Factor 4 Mops Up Heparin at Sites of Endothelial Damage
1218(1)
Plasminogen Activators
1218(1)
Adhesive Secretions of Endothelium
1218(3)
SELECTINS AND THEIR CARBOHYDRATE LIGANDS
1218(1)
P-Selectin Is the Adhesion Receptor for Macrophages
1219(1)
INTEGRINS CAN READ THE RGD ZIP CODE
1220(1)
IMMUNOGLOBULIN LIGANDS FOR INTEGRINS
1220(1)
Endothelial Activation During Inflammation: Another Link Between Coagulation and Cytokine Cascades
1220(1)
von Willebrand Factor (vWF): The Adhesive Protein Resistant to Shear
1221(2)
STRUCTURE AND FUNCTION OF VWF
1221(1)
vWF Is the Prototype Multifunctional, Multidomain Adhesion Molecule
1221(1)
vWF Forms Multimeric Velcrolike Grippers
1221(1)
Storage and Secretion of vWF
1221(1)
Platelets Have More Than One Binding Site for vWF: the IIb-IIIa Complex
1222(1)
VWF IS THE CARRIER OF FACTOR VIII
1222(1)
Vascular Response to Deeper Injury
1223(1)
Fibronectin Is a Subendothelial Glue
1223(1)
Loss of Endothelium
1223(1)
PLATELETS AND FORMATION OF THE HEMOSTATIC PLUG
1224(14)
Platelet Anatomy and Contents
1224(4)
Membrane Lipids
1224(1)
PLATELET MEMBRANE GLYCOPROTEINS MEDIATE ADHESION
1224(2)
Glycoprotein Ia-IIa
1225(1)
Glycoprotein IIb-IIIa
1225(1)
Ib-IX: vWF as Seen from Both Sides Now
1225(1)
THE SUBMEMBRANE CONTAINS MOTOR FILAMENTS
1226(1)
Activated Platelets Are Capable of Directional Movement
1226(1)
Platelet Contraction
1226(1)
Platelet Activating Factor (PAF)
1226(1)
PLATELET SHAPE CHANGES
1227(1)
XXX-Granules Secrete Adhesive Proteins
1228(1)
Platelet Adhesion, Aggregation, and Secretory Activity
1228(6)
ADHESION
1228(1)
Adherent Platelets Form a Pavement
1229(1)
PLATELET AGGREGATION
1229(1)
STICKINESS OF ACTIVATED PLATELETS IS CAUSED BY FIBRINOGEN BINDING
1230(1)
Platelet Recognition Sites on Fibrinogen
1230(1)
vWF Contributes to Platelet Stickiness
1231(1)
PLATELET ACTIVATION BY AGONISTS
1231(1)
ADP
1231(1)
Thrombin
1231(1)
Epinephrine
1231(1)
Collagen
1231(1)
Thrombospondin Strengthens Binding of Fibrinogen to Platelets
1231(1)
INTRACELLULAR REGULATION OF XXX(IIb)XXX(3) ANCHORAGE FUNCTION
1232(1)
Outside-In Signaling
1232(1)
PLATELET SECRETION: STIMULUS-RESPONSE COUPLING IN ACTIVATED PLATELETS
1232(1)
COMPARTMENTALIZATION OF ADENINE NUCLEOTIDES
1232(1)
The Cytoplasmic Compartment
1232(1)
ADP Bound to F-Actin
1232(1)
Dense Granule ADP
1232(1)
PRODUCTION OF EICOSANOIDS
1233(1)
Lipoxygenase Pathway: A Dead End?
1233(1)
THE CYCLOOXYGENASE PATHWAY GENERATES THROMBOXANE AND IS INHIBITED BY ASPIRIN
1233(1)
INHIBITION OF PLATELET STIMULATION BY CAMP AND PGI2: THE PLOT (NOT THE CLOT) THAT THICKENS
1233(1)
Baby Aspirin: A Half-told Story
1234(1)
Phosphoinositide System
1234(2)
PLATELET-DERIVED GROWTH FACTOR: THE WOUND HORMONE
1235(1)
Platelet Procoagulant Activity
1236(2)
THE PROTHROMBINASE COMPLEX
1236(1)
Factor Va Is the Docking Site for Factor Xa: A Recap
1236(1)
THE FACTOR VIII COMPLEX
1236(1)
The Importance of Being a Surface
1237(1)
ACTIVATION OF PROTHROMBIN RECAPITULATED
1237(1)
THROMBIN RECEPTORS
1237(1)
COAGULATION
1238(14)
WHY THE COMPLEXITY?
1238(1)
Coagulation Cascade Revised
1239(1)
ACTIVATION OF FACTOR IX BY THE EXTRINSIC PATHWAY
1240(1)
Coagulation Is Initiated by Factor VIIa-Tissue Factor
1240(3)
TISSUE FACTOR
1240(1)
FACTOR VII
1241(1)
TISSUE FACTOR PATHWAY INHIBITOR
1241(1)
Mechanism of Action of TFPI
1241(2)
Plasma Levels of TFPI in Health and Disease
1243(1)
BLOOD COAGULATION PATHWAYS: THE NEW LOOK REPRISED
1243(1)
Fibrinogen and Fibrin
1243(2)
Ultrastructural Appearance of Fibrinogen
1243(1)
BIOSYNTHESIS OF FIBRINOGEN
1243(1)
Gene Coding of Fibrinogen Synthesis
1244(1)
THROMBIN CONVERTS FIBRINOGEN TO FIBRIN
1244(1)
FIBRIN MONOMERS POLYMERIZE TO FORM DOUBLE-STRANDED PROTOFIBRILS
1244(1)
FIBRIN FIBRILS BECOME COVALENTLY CROSSLINKED BY FACTOR XIII
1245(1)
Fibronectin Is Also Crosslinked to Fibrin by Factor XIIIa
1245(1)
DISMANTLING OF THE FIBRIN CLOT BY PLASMIN
1245(1)
Degradation of Fibrin
1245(1)
Domain Structures of Coagulation Proteins
1245(1)
Prothrombin (Factor II) and Thrombin
1246(3)
VITAMIN K-DEPENDENT PROTEINS CONTAIN 7 TO 12 N-TERMINAL GLA RESIDUES
1246(2)
Metabolism of the Signal Sequence
1246(1)
XXX-Carboxylation and Epoxidation
1247(1)
Vitamin K Antagonists Block Synthesis of Prothrombin and Factors VII, IX, and X
1247(1)
ACTIVATION OF PROTHROMBIN TO THROMBIN BY STEPWISE PROTEOLYSIS
1248(1)
Thrombin Activation Is Self-extinguishing
1248(1)
Thrombin Is the Master Enzyme of Coagulation
1248(1)
Factor X
1249(1)
Physical Properties
1249(1)
Activation of Factor X
1249(1)
Factor IX
1249(1)
Activation of Factor IX
1249(1)
Factor IXa Activates Factor X
1249(1)
Factor VII
1250(1)
Cofactors of the Clotting Cascade: Factors V and VIII
1250(1)
FACTOR V
1250(1)
The Heavy Chain of Factor Va Is the Receptor for Factor Xa
1250(1)
FACTOR VIII IS CARRIED BY VWF AND IS ESSENTIAL TO FACTOR X ACTIVATION
1250(1)
Factor VIII Is an Essential Cofactor in Factor X Activation
1250(1)
Factor XIII (Fibrin Stabilizing Factor)
1250(1)
Contact Activation Complex: Factor XII, Prekallikrein, HMWK, and Factor XI
1251(1)
INITIATION OF CONTACT REACTIONS
1251(1)
Plasma Kallikrein-HMWK-Bradykinin System
1251(1)
AMPLIFICATION OF THE CONTACT SYSTEM: ACTIVATION OF FACTOR IX
1251(1)
Regulation of the Contact System
1251(1)
CONTACT PHASE PARTICIPATES IN INFLAMMATION, NOT HEMOSTASIS
1251(1)
FIBRINOLYSIS AND FIBRIN DEGRADATION
1252(1)
Plasmin Is Generated by Plasminogen Activators
1252(1)
PLASMIN ACTIVITY IS DIVERTED BY PROTEASE INHIBITORS
1252(1)
PAI-1 Levels and Thrombosis
1252(1)
Synthesis and Stabilization of PAI-1
1252(1)
Circadian Rhythms and Myocardial Infarction
1252(1)
COMPONENTS OF THE FIBRINOLYTIC SYSTEM
1253(4)
Plasminogen Activators
1253(2)
TISSUE TYPE PLASMINOGEN ACTIVATOR (t-PA)
1253(1)
Annexin II Is the Receptor for Both t-PA and Plasmin
1254(1)
UROKINASE TYPE PLASMINOGEN ACTIVATOR (U-PA)
1254(1)
No Finger, One Kringle, No Fibrin Binding
1254(1)
STREPTOKINASE AND APSAC
1255(1)
Staphylokinase
1255(1)
Plasminogen and Plasmin
1255(2)
CONVERSION TO PLASMIN
1255(1)
Gene Structure and Genetic Polymorphisms
1255(1)
INHIBITORS OF PLASMIN: XXX2-ANTIPLASMIN
1255(1)
FIBRIN DEGRADATION, CLOT LYSIS, AND "SPLIT PRODUCTS"
1256(1)
DIGESTION OF FIBRINOGEN AND FIBRIN MONOMERS
1256(1)
Digestion of Crosslinked Fibrin
1256(1)
Fibrin Degradation Products Inhibit Coagulation
1256(1)
MEASURING FIBRINOLYSIS
1257(1)
Detection of Fibrin Degradation Products
1257(1)
Elevated D Dimer Levels Are the Insignia of DIC
1257(1)
TESTS OF HEMOSTASIS
1257(20)
Tests of Primary Hemostasis
1257(1)
PLATELET COUNT
1258(1)
BLEEDING TIME
1258(1)
Tests of Secondary Hemostasis
1258(19)
TESTING CLOTTING PATHWAYS
1258(1)
Activated Partial Thromboplastin Time (APTT)
1258(1)
Prothrombin Time (Thromboplastin Time)
1258(1)
Thrombin Time
1258(1)
Inhibitor Screen
1258(1)
DEFICIENCIES OF SPECIFIC FACTORS
1258(1)
Fibrinogen
1258(1)
Factor XIII
1258(1)
Other Specific Factors
1259(1)
COAGULATION TESTING IS BEING AUTOMATED
1259(1)
30. Vascular Purpuras 1277(24)
Angiogenesis
1277(1)
ANGIOGENIC FACTORS: DEGRADERS, GROWERS, AND REGULATORS
1277(1)
Heparin Binding Growth Factors
1277(1)
Platelet-Derived Growth Factor (PDGF)
1277(1)
VASCULAR MALFORMATIONS
1278(6)
Hereditary Hemorrhagic Telangiectasia
1278(2)
PATHOGENESIS
1278(1)
DESCRIPTION
1278(1)
Associated Vascular Anomalies
1279(1)
TREATMENT
1279(1)
Cavernous Hemangiomas and Hemangioendotheliomas
1280(1)
Proliferative Vascular Malformations
1280(2)
KASABACH-MERRITT SYNDROME
1280(2)
Therapy
1281(1)
HEMANGIOENDOTHELIOMAS
1282(1)
Acquired Proliferative Hemangiomas Simulate Hereditary Varieties
1282(2)
Chemically Induced Hemangiosarcomas
1282(1)
KAPOSI'S SARCOMA
1282(2)
A Cytokine Cybercrime
1283(1)
HEREDITARY DISORDERS OF CONNECTIVE TISSUE
1284(1)
Collagen Holds Us Together
1284(1)
EHLERS-DANLOS SYNDROME
1284(1)
PSEUDOXANTHOMA ELASTICUM
1284(1)
OSTEOGENESIS IMPERFECTA
1285(1)
MARFAN'S SYNDROME
1285(1)
FABRY'S DISEASE
1285(1)
ACQUIRED VASCULAR PURPURAS
1285(16)
Mechanical Purpura
1285(5)
AGE-RELATED VASCULAR LESIONS
1286(1)
Cherry or Senile Angiomas
1286(1)
Venous Lakes
1286(1)
Senile Telangiectasia
1286(1)
Senile Purpura
1286(1)
Angiodysplasia of the Elderly
1286(1)
SCURVY
1287(1)
Hemorrhagic Manifestations
1287(1)
AMYLOIDOSIS
1288(1)
Deficiencies of Factor X and Other Vitamin K-Dependent Clotting Factors in Amyloidosis
1288(1)
FACTITIAL PURPURA: PSYCHOGENIC BLEEDING
1289(1)
Abuse
1289(1)
Self-Abuse
1289(1)
Autoerythrocyte Sensitization
1289(1)
Autosensitivity to DNA
1290(1)
Religious Stigmata
1290(1)
Pleasant Forms of Mechanical Purpura
1290(1)
Vascular Purpura Caused by Infections, Drugs, or Chemicals
1290(1)
PURPURA ASSOCIATED WITH INFECTIONS
1290(1)
PURPURA CAUSED BY DRUGS OR CHEMICALS
1291(1)
Vasculitis (Palpable Purpura)
1291(10)
MOST VASCULITIS SYNDROMES REPRESENT IMMUNE COMPLEX DISEASE
1291(1)
VASCULITIS SYNDROMES
1291(1)
Description
1291(1)
Management
1291(1)
HENOCH-SCHONLEIN PURPURA
1292(9)
Differentiation of Henoch-Schonlein Purpura from Leukocytoclastic Vasculitis
1292(1)
Description
1292(1)
Gastrointestinal Manifestations
1293(1)
Henoch-Schonlein Nephritis Is an IgA Nephropathy
1293(1)
Management
1294(1)
31. Disorders of Platelets 1301(60)
MEGAKARYOCYTE DEVELOPMENT AND PLATELET RELEASE
1301(6)
MEGAKARYOCYTE MATURATION
1301(1)
POLYPLOID MEGAKARYOCYTES
1302(1)
Humoral Regulation of Thrombopoiesis
1302(1)
IL-11, the Megakaryocyte Colony Stimulating Cytokine
1303(1)
REGULATION OF MEGAKARYOCYTE MATURATION: THROMBOPOIETIN AND ITS MPL RECEPTOR
1303(1)
INHIBITORS OF MEGAKARYOCYTE PRODUCTION
1303(1)
Thrombocytopoiesis
1303(2)
PLATELETS ARE RELEASED BY MEGAKARYOCYTE FRAGMENTATION
1304(1)
Determinants of Platelet Size
1304(1)
Platelet Durability Reflects Its Rugged Cytoskeleton
1304(1)
Kinetics of Platelet Production and Destruction
1305(2)
PLATELETS DISTRIBUTION
1305(1)
PLATELET LIFESPAN
1306(1)
THROMBOCYTOPENIA DUE TO DEFECTIVE PLATELET PRODUCTION
1307(3)
Pseudothrombocytopenia
1307(1)
Hereditary Thrombocytopenia
1307(2)
AMEGAKARYOCYTIC THROMBOCYTOPENIA AND THE TAR SYNDROME
1307(1)
X-Linked Amegakaryocytic Thrombocytopenia
1307(1)
WISKOTT-ALDRICH SYNDROME
1308(1)
Therapy
1308(1)
MAY-HEGGLIN ANOMALY
1308(1)
HEREDITARY MACROTHROMBOCYTOPENIA
1308(1)
Acquired Amegakaryocytic Thrombocytopenic Purpura
1309(1)
DRUG-INDUCED MEGAKARYOCYTE HYPOPLASIA
1309(1)
Chlorothiazide and Its Congeners
1309(1)
Drug-Induced Thrombocytopenia of the Newborn
1309(1)
Alcohol
1309(1)
Estrogens, Prednisone, and Interferon
1310(1)
VIRAL INFECTIONS
1310(1)
CYCLIC THROMBOCYTOPENIA
1310(1)
THROMBOCYTOPENIA DUE TO INCREASED PLATELET DESTRUCTION
1310(21)
Immune Thrombocytopenia (ITP)
1310(4)
ITP SHOULD STAND FOR IMMUNE THROMBOCYTOPENIC PURPURA
1311(1)
PLATELET SURFACE IgG
1312(1)
GLYCOPROTEIN TARGETS FOR ANTIBODIES IN ITP SERA
1312(1)
IgG ANTIBODY-COATED PLATELETS ARE TRAPPED AND DESTROYED BY MONOCYTES AND MACROPHAGES
1312(1)
Immune Thrombocytopenia Involving IgM and Complement
1312(1)
PLATELET ANTIGENS
1313(1)
Platelet-Specific Antigens
1313(1)
HLA and ABH Antigens
1313(1)
PLATELET Fc RECEPTORS BIND ANTIGEN-ANTIBODY COMPLEXES
1313(1)
ACUTE ITP AND CHRONIC ITP DIFFER IN PATHOGENESIS
1313(1)
Acute ITP
1314(2)
DESCRIPTION
1314(2)
Acute ITP May Be Caused by Antibodies to Platelet-Adsorbed Viral Antigens
1314(1)
Therapy
1315(1)
Platelet Transfusions, Prednisone, and High-Dose Intravenous Gamma Globulin
1315(1)
But How Does It Work?
1316(1)
Anti-D for Treatment of ITP: A Fiasco
1316(1)
Chronic ITP
1316(6)
CHRONIC ITP IS CAUSED BY ANTIPLATELET ANTIBODIES
1316(2)
HIV-Related Thrombocytopenia
1317(1)
HIV-Induced Megakaryocyte Injury
1317(1)
IgG Antiplatelet Antibodies Sometimes Suppress Megakaryocytopoiesis
1317(1)
Autoantibody Production Originates in the Spleen
1317(1)
THERAPY AND PROGNOSIS
1318(1)
Corticosteroids
1318(1)
Primum Non Nocere
1318(1)
SPLENECTOMY
1318(1)
Splenic Irradiation
1319(1)
ITP Spleens Contain Foamy Macrophages, Sea Blue Histiocytes, and Florid Vascularity
1319(1)
TREATMENT OF REFRACTORY CHRONIC ITP
1319(1)
High-Dose Gamma Globulin Concentrates
1319(1)
PULSED HIGH-DOSE DEXAMETHASONE: THE MIRACLE CURE?
1320(1)
NONMIRACULOUS MEASURES
1320(1)
Immunosuppression
1320(1)
Danazol
1321(1)
Other Failed Therapies
1321(1)
NEONATAL ITP
1321(1)
Reacting and Overreacting to Gestational ITP
1321(1)
NEONATAL ITP VERSUS NEONATAL ALLOIMMUNE THROMBOCYTOPENIA
1321(1)
Novel Alloantigens of GPIIIa Provoke Most Neonatal Alloimmune Purpuras
1321(1)
Posttransfusion Purpura: Berserk Alloantibodies
1322(1)
The PTP Enigma
1322(1)
Description
1323(1)
TREATMENT
1323(1)
Recurrence
1323(1)
Drug-Induced Immune Thrombocytopenia
1323(4)
Drugs Identified as Causes of Immune Thrombocytopenia: The Quinine-Quinidine Model
1323(1)
QUININE- AND QUINIDINE-DEPENDENT THROMBOCYTOPENIA
1324(1)
Pathogenesis
1324(1)
Recovery Begins as PA IgG Levels Return to Normal
1324(1)
Diagnostic Confirmation In Vitro and In Vivo
1325(1)
Treatment
1325(1)
HEPARIN-INDUCED THROMBOCYTOPENIA AND THROMBOSIS
1325(2)
Pathogenesis of HIT Syndrome
1326(1)
Heparin Binding and Platelet Fragmentation
1326(1)
Diagnosis, Prevention, and Management of HIT Syndrome. The Hirudin Option
1327(1)
OTHER DRUGS COMMONLY ASSOCIATED WITH ANTIBODY-MEDIATED THROMBOCYTOPENIA
1327(1)
Gold
1327(1)
Trimethoprim-Sulfamethoxazole
1327(1)
Platelet Consumption Disorders
1327(4)
THROMBOTIC THROMBOCYTOPENIC PURPURA
1327(4)
Pathogenesis
1328(1)
Attacks of TTP Are Triggered by Endothelial Release of Unusually Large XXXWF Multimers
1329(1)
Description
1329(1)
Prognosis and Treatment: Plasma Therapy
1330(1)
Plasma Exchange Achieves Superior and More Durable Remissions
1330(1)
HEMOLYTIC-UREMIC SYNDROME
1331(1)
THROMBOCYTOPENIA CAUSED BY EXCESSIVE SPLENIC POOLING
1331(1)
THROMBOCYTHEMIA
1331(1)
HEREDITARY QUALITATIVE DISORDERS OF PLATELETS
1331(7)
Defects in Platelet-Agonist Interaction: Receptor Defects
1332(1)
Bernard-Soulier (Giant Platelet) Syndrome
1333(1)
PATHOGENESIS
1333(1)
BSS Platelets Fail to Adhere to Subendothelium for Lack of the GPIb-IX-V Complex, Locus of the vWF Receptor
1333(1)
TREATMENT
1333(1)
Glanzmann's Thrombasthenia
1334(1)
Thrombasthenic Platelets Respond to Agonists but Fail to Aggregate
1334(1)
PATHOGENESIS: THROMBASTHENIC PLATELETS LACK SUFFICIENT FIBRINOGEN RECEPTORS
1334(1)
MANAGEMENT
1335(1)
Hereditary Abnormalities of Platelet Secretion
1335(3)
DENSE-BODY DEFICIENCY (XXX-SPD) AND XXX STORAGE POOL DISEASE
1335(2)
XXX-SPD
1336(1)
How Full Are the Dense Granule Sacs?
1336(1)
XXX-SPD Is Sometimes Associated with Other Inherited Disorders
1336(1)
XXX-SPD
1337(1)
XXX-SPD (GRAY PLATELET SYNDROME)
1337(1)
XXX-SPD Is a Packaging Defect
1337(1)
DEFECTS OF THROMBOXANE SYNTHESIS
1337(1)
Cyclooxygenase Deficiency
1337(1)
Thromboxane Synthetase Deficiency
1338(1)
Thromboxane A(2) Insensitivity
1338(1)
MISCELLANEOUS HEREDITARY DISORDERS OF PLATELET FUNCTION
1338(1)
Montreal Platelet Syndrome
1338(1)
Epstein's Syndrome
1338(1)
PLATELET SECRETION DEFECTS WITH NORMAL GRANULE STORES AND NORMAL THROMBOXANE SYNTHESIS
1338(1)
DEFECTIVE PLATELET COAGULANT ACTIVITY: SCOTT SYNDROME
1338(1)
ACQUIRED QUALITATIVE DISORDERS OF PLATELET FUNCTION
1338(4)
Uremia
1338(1)
DESCRIPTION AND PATHOGENESIS
1338(1)
Uremic Bleeding Results from Impaired Platelet Interaction with Endothelium
1339(1)
THERAPY: DIALYSIS, DDAVP, AND RENAL TRANSPLANTATION
1339(1)
Chronic Myeloproliferative Disorders
1339(1)
Acquired Storage Pool Disorders
1339(1)
PLATELET DYSFUNCTION IN MYELODYSPLASTIC AND MYELOPROLIFERATIVE DISORDERS
1339(1)
Cardiopulmonary Bypass
1340(1)
Drugs That Inhibit Platelet Function
1340(2)
ASPIRIN
1340(2)
Aspirin-Exacerbated Bleeding Disorders
1341(1)
Adenylate Cyclase Activators and Phosphodiesterase Inhibitors
1341(1)
ANTIMICROBIAL AGENTS
1342(1)
DEXTRANS
1342(1)
ETHANOL
1342(1)
PLATELETS CAN GIVE ONE A HEADACHE
1342(1)
PLATELET TRANSFUSION THERAPY
1342(19)
RANDOM DONOR PLATELET CONCENTRATES
1342(1)
PLATELET STORAGE
1342(19)
Frozen Platelets: A Fixture of Transplant Management
1343(1)
Assessment of Platelet Recovery
1343(1)
Alloimmunization: The Bugbear of Platelet Transfusion
1343(1)
Managing Refractoriness in Platelet-Dependent Thrombocytopenia
1344(1)
Single Donor Platelet Apheresis
1344(1)
Risks of Platelet Transfusions
1344(1)
32. Disorders of Coagulation 1361(54)
COMMON HEREDITARY DISORDERS OF COAGULATION
1361(1)
HEMOPHILIA A
1361(15)
Incidence and Prevalence
1361(1)
Inheritance of Hemophilia A
1361(2)
ABOUT 15% OF HEMOPHILIA CASES ARE SPORADIC AND HALF OF ALL CASES REFLECT RECENT MUTATIONS
1362(1)
Molecular Genetics of Hemophilia A
1363(2)
MUTATIONS ARE HETEROGENEOUS AND FAMILY SPECIFIC
1363(1)
Most Hemophilia Is Caused by Inheritance of a Single Nucleotide Substitution
1364(1)
CARRIER DETECTION AND PRENATAL DIAGNOSIS
1364(1)
Prenatal Diagnosis
1364(1)
Acceptance of Genetic Counseling
1365(1)
Pathogenesis
1365(1)
vWF Is the Carrier Protein for VIII:C
1365(1)
DELAYED BLEEDING IN HEMOPHILIA A
1365(1)
Description
1366(5)
PSYCHOLOGICAL AND SOCIAL EFFECTS
1366(1)
Family Adaptation
1366(1)
HEMARTHROSES
1366(2)
Chronic Hemophilic Arthropathy
1367(1)
MUSCLE BLEEDING AND HEMATOMA
1368(1)
Iliopsoas Hematoma
1368(1)
Sublingual Hematoma
1368(1)
BLOOD CYSTS AND PSEUDOTUMORS
1369(1)
RENAL TRACT ABNORMALITIES
1369(1)
INTRACRANIAL BLEEDING
1369(1)
EPISTAXIS AND BLEEDING FROM OTHER MUCOUS MEMBRANES
1370(1)
Stress, Peptic Ulceration, and Gastrointestinal Bleeding
1370(1)
Aspirin Is Taboo
1370(1)
DENTAL EXTRACTIONS
1370(1)
Curb the Exploring Tongue
1370(1)
Replacement Therapy
1371(5)
THERAPEUTIC MATERIALS
1371(2)
Virucidal Processing of Factor VIII: Success in Purging HIV
1373(1)
Recombinant Factor VIII
1373(1)
ANTIBODY INHIBITORS OF FACTOR VIII
1373(1)
Circumvention of Factor VIII Inhibitors
1373(1)
Substitution of Porcine Factor VIII
1373(1)
Prothrombin Complex Concentrates Bypass Factor VIII Requirements
1374(1)
Purified Factor VIIa
1374(1)
Extracorporeal Adsorption of Antibody
1374(1)
DDAVP: An Alternative Therapy for Mild Hemophilia (and a Boon to Blood Bankers)
1374(1)
REPLACEMENT THERAPY IN HEMOPHILIA UNCOMPLICATED BY INHIBITORS
1374(2)
On-Demand Therapy
1374(1)
Prophylaxis
1375(1)
Comprehensive Management and Self-Therapy
1375(1)
The Payoff from Comprehensive Care Programs
1375(1)
Postscripts on Hemophilia A: Liver Transplantation, Gene Therapy, and Back to the Future
1376(1)
HEMOPHILIA B
1376(6)
Inheritance of Hemophilia B
1377(1)
Classification and Molecular Genetics
1377(4)
HEMOPHILIA B PHENOTYPE IS EXCEPTIONALLY HETEROGENEOUS
1377(1)
REPRESENTATIVE GENETIC VARIANTS OF HEMOPHILIA B
1378(1)
Mutations in the Promoter Region
1378(1)
Mutations in the Propeptide Region: A Tale of a Tail
1378(1)
Mutations in the Gla Domain Block Binding to Phospholipids
1379(1)
Mutations in the Epidermal Growth Factor (EGF)-Like Domain
1379(1)
Mutations in the Activation Peptide Cause Unbalanced Cleavage
1379(1)
Mutations in or near the Catalytic Domain of the Heavy Chain Block Substrate Binding
1379(1)
Gross Deletions or Insertions Cause Severe Hemophilia B
1379(1)
CARRIER DETECTION AND GENETIC SCREENING
1379(2)
Description
1381(1)
Replacement Therapy
1381(1)
Prothrombin Complex Concentrates (PCC)
1381(1)
COMPLICATIONS OF FACTOR IX CONCENTRATE THERAPY
1381(1)
Thrombotic Complications
1382(1)
Inhibitors (Antibodies) to Factor IX Are Uncommon
1382(1)
Gene Therapy of Hemophilia B
1382(1)
VON WILLEBRAND'S DISEASE
1382(9)
Inheritance of vWD
1382(1)
vWF Gene Resides on Chromosome 12
1383(1)
Pathogenesis of vWD Reprised
1383(1)
vWF IS THE CARRIER PROTEIN FOR FACTOR VIII
1383(1)
vWF Mediates Platelet Adherence to Subendothelium
1383(1)
Classification and Molecular Defects in vWD
1384(5)
CLASSIFICATION
1384(1)
Type IA vWD
1385(1)
Type IB
1385(1)
Type IC
1385(1)
Type II vWD
1385(3)
Type IIA
1385(2)
Type IIB
1387(1)
Type IIC
1387(1)
Type IID
1388(1)
Type III vWD
1388(1)
Platelet Type (Pseudo) vWD
1388(1)
Variants with Impaired Factor VIII Binding: XXXWD Normandy
1388(1)
PRENATAL DIAGNOSIS AND GENETIC COUNSELING IN VWD
1388(1)
Cave Adsum!
1388(1)
Acquired vWD
1389(1)
Description
1389(1)
Epistaxes, Gingival Bleeding, Easy Bruising, and Menorrhagia
1389(1)
Therapy
1389(2)
DDAVP PROVIDES TEMPORARY RELIEF IN TYPE I VWD
1389(1)
DDAVP as an Adjunct to Cardiac Surgery in Patients Without vWD
1390(1)
Estrogens
1390(1)
vWF REPLACEMENT THERAPY
1390(1)
vWF Induces Factor VIII Synthesis in vWD But Not in Hemophilia
1390(1)
Anti-vWF Antibodies
1391(1)
Management of Gingival Bleeding
1391(1)
RARE HEREDITARY DISORDERS OF COAGULATION
1391(7)
Fibrinogen Defects
1391(2)
AFIBRINOGENEMIA
1392
Description
1392
Therapy
1392
HYPOFIBRINOGENEMIA
1392
Dysfibrinogenemia
1393(1)
MOLECULAR PATHOLOGY AND CLASSIFICATION OF FIBRINOGEN VARIANTS
1393(1)
Classification
1393(1)
Abnormal Fibrinopeptide Release
1393(1)
Polymerization Defects
1393(1)
The Odd Couples
1393(1)
Stabilization Defects
1394(1)
DESCRIPTION
1394(1)
THERAPY
1394(1)
Hereditary Deficiency of Factor XIII
1394(1)
DESCRIPTION
1394(1)
DIAGNOSIS AND TREATMENT
1395(1)
Hereditary Deficiency of Factor XI
1395(1)
DESCRIPTION
1395
DIAGNOSIS AND TREATMENT
1395
Hereditary Disorders Prolonging the Prothrombin Time
1395(2)
HEREDITARY PROTHROMBIN DEFICIENCY
1396(1)
Dysprothrombinemias
1396(1)
Diagnosis and Treatment
1396(1)
HEREDITARY FACTOR V DEFICIENCY
1396(1)
Description
1396(1)
Diagnosis and Treatment
1396(1)
HEREDITARY FACTOR VII DEFICIENCY
1396(1)
Dysproconvertinemias
1396(1)
Diagnosis and Treatment
1397(1)
HEREDITARY DEFICIENCY OF FACTOR X
1397(1)
Factor X Variants
1397(1)
Treatment
1397(1)
Deficiencies of Factor XII, Prekallikrein, and High Molecular Weight Kininogen
1397(1)
Hereditary Deficiencies of Fibrinolytic Inhibitors
1397(1)
XXX(2)-ANTIPLASMIN DEFICIENCY
1397(1)
Diagnosis and Treatment
1397(1)
COMBINED DEFECTS
1398(1)
ACQUIRED DISORDERS OF COAGULATION
1398(17)
Disorders of Vitamin K-Dependent Coagulation Factors
1398(2)
VITAMIN K DEFICIENCY
1398(1)
Diagnosis and Therapy
1398(1)
Hemorrhagic Disease of the Newborn
1398(1)
Vitamin K Deficiency in Liver Disease
1398(1)
Acquired Dysfibrinogenemia in Liver Disease
1399(1)
DRUG-INDUCED VITAMIN K DEFICIENCY
1399(1)
Broad Spectrum Antibiotics
1400(1)
Acquired Anticoagulants
1400(15)
FACTOR VIII INHIBITORS IN PATIENTS WITHOUT HEMOPHILIA
1401(14)
Management
1401(1)
Factor IX Inhibitors
1401(1)
Factor V Inhibitors
1401(1)
Factor XIII Inhibitors
1401(1)
vWF Inhibitors
1401(14)
33. Thrombotic Disorders 1415(46)
PATHOGENESIS OF ARTERIAL AND VENOUS THROMBOSIS
1415(3)
Arterial Thrombosis
1415(1)
ACUTE CARDIOEMBOLIC STROKE
1415(1)
Venous Thrombosis and Thromboembolism
1415(3)
STASIS, RHEOLOGY, AND DEEP VEIN THROMBOSIS
1416(1)
Propagation
1417(1)
FATE OF THROMBI
1417(1)
Plasmin: Plasminogen Activator and Its Inhibitor
1417(1)
EVALUATION OF INCREASED THROMBOTIC RISK
1418(1)
DESCRIPTION AND DIAGNOSIS OF VENOUS THROMBOSIS AND PULMONARY EMBOLISM
1418(5)
Clinical Presentation
1418(5)
D Dimer Assays
1419(1)
METHODS FOR IMAGING AND STAGING THROMBI
1419(2)
Contrast Venography
1419(1)
CT Scanning
1419(1)
MR Imaging
1419(1)
Imaging with Thrombus-Avid Radiopharmaceuticals
1420(1)
(111)In-Labeled Platelets
1420(1)
(99m)Tc-Labeled Plasmin Scintigraphy
1420(1)
Ultrasonography
1420(1)
Doppler Ultrasonography Measures Blood Flow
1420(1)
Sound Judgment: Duplex Venous Scanning
1420(1)
Breaking the Sound Barrier: Intravascular Imaging
1421(1)
HEMODYNAMIC MEASUREMENTS IN DEEP VENOUS THROMBOSIS
1421(1)
Impedance Plethysmography
1421(1)
Thermography
1422(1)
PULMONARY EMBOLISM
1422(1)
Lung Scans
1422(1)
Pulmonary Angiography
1422(1)
HEREDITARY THROMBOTIC DISORDERS
1423(6)
Antithrombin Deficiency
1423(2)
PATHOPHYSIOLOGY AND DIAGNOSIS
1423(1)
Molecular Pathology: AT Variants
1423(1)
Antithrombin-TRI
1424(1)
Differential Diagnosis
1424(1)
DESCRIPTION
1424(1)
MANAGEMENT
1424(1)
Heparin Plus Stanozolol (Stanazol)
1425(1)
A Damp Blanket
1425(1)
HEPARIN COFACTOR II DEFICIENCY
1425(1)
Acquired Deficiency of Heparin Cofactor II
1425(1)
Protein C Deficiency
1425(2)
PATHOPHYSIOLOGY
1425(1)
Molecular Pathology
1426(1)
DESCRIPTION
1426(1)
Venous Thrombosis
1426(1)
Warfarin-Induced Skin Necrosis
1426(1)
Neonatal Purpura Fulminans
1426(1)
MANAGEMENT
1426(1)
Protein S Deficiency
1427(1)
PROTEIN S HAS DUAL RESPONSIBILITIES
1427(1)
Molecular Pathology: Confusion Compounded
1427(1)
DESCRIPTION AND MANAGEMENT
1428(1)
Acquired Protein S Deficiency
1428(1)
Hereditary Resistance to Activated Protein C
1428(1)
Why the Singularity of the Arg(506)XXXGln Mutation?
1429(1)
Other Hereditary Causes of APC Resistance Are Vanishingly Rare
1429(1)
Dysfibrinogenemias and Thromboembolic Disease
1429(1)
Homocystinuria
1429(1)
ACQUIRED PRETHROMBOTIC DISORDERS
1429(4)
COAGULATION AND CANCER: THROMBOTIC PREMONITIONS
1430(1)
SURGERY, TRAUMA, AND THE POSTPARTUM PERIOD
1430(1)
The Postpartum Period, Not Pregnancy, Is Thrombogenic
1431(1)
Oral Contraceptive Pills: The Endless Debate
1431(1)
Antiphospholipid Syndrome and Lupus Anticoagulants
1431(2)
LUPUS ANTICOAGULANTS
1431(1)
The Lupus Anticoagulant Paradox
1432(1)
DESCRIPTION
1432(1)
APLS and Pregnancy
1432(1)
Early Detection and Therapy
1433(1)
INR, PTR, and ISI Should Be Learned PDQ
1433(1)
Lupus Anticoagulant-Acquired Hypoprothrombinemia Syndrome
1433(1)
PROPHYLAXIS AND TREATMENT OF THROMBOTIC DISORDERS
1433(7)
Prophylaxis
1433(2)
DRUGS THAT ALTER PLATELET FUNCTION
1433(1)
ASPIRIN
1434(1)
DEXTRAN
1434(1)
DRUGS THAT ALTER COAGULATION
1434(1)
Oral Anticoagulants
1434(1)
The Shifting Guidelines for Safe Anticoagulation
1434(1)
Low-Dose Heparin Prophylaxis
1434(1)
The Advantages of Low Molecular Weight Heparin Preparations
1435(1)
Complications and Control of Low-Dose Heparin Prophylaxis
1435(1)
Treatment Strategy in Thromboembolic Disorders
1435(1)
FULL-DOSE INTRAVENOUS HEPARIN THERAPY
1435(1)
POSTHEPARIN MAINTENANCE WITH WARFARIN
1435(1)
Intermediate-Dose Heparin plus Warfarin
1436(1)
ALTERNATIVE MEDICAL AND SURGICAL PROCEDURES
1436(1)
Antiplatelet Drug Combinations. Anabolic Steroids
1436(1)
Surgical Prevention of Pulmonary Embolism: The Caval Umbrella Filter
1436(1)
MANAGEMENT OF HEPARIN-INDUCED BLEEDING
1436(1)
Ancrod
1436(1)
Thrombolytic Therapy
1436(4)
UK and SK Dissolve Pulmonary Emboli If Given Promptly
1437(1)
WITH CARDIOVASCULAR DISEASES STAKES ARE HIGH
1438(1)
COMPARATIVE EFFICACY OF THROMBOLYTIC AGENTS IN ACUTE MYOCARDIAL INFARCTION
1438(1)
Longterm Survival Comparisons Give Only a Slight Edge to rt-PA
1439(1)
INTRA-ARTERIAL THROMBOLYSIS FOR ISCHEMIC STROKE: ONE SMALL STEP FOR MANKIND
1439(1)
Adjunctive Therapy for Thrombolysis: Aspirin and Hirudin
1439(1)
TREATMENT OF PERIPHERAL ARTERIAL DISEASE: CARPE DIEM
1440(1)
DISSEMINATED INTRAVASCULAR COAGULATION
1440(21)
Pathogenesis
1440(2)
CIRCULATING CROSSLINKED FIBRIN DERIVATIVES ARE SPECIFIC MARKERS FOR DIC
1440(1)
Mechanism of Bleeding in DIC
1441(1)
CONSUMPTION OF COAGULATION FACTORS AND PLATELETS
1441(1)
Secondary Fibrinolysis Versus Primary Microthrombosis
1441(1)
Description and Management of DIC Syndromes
1442(3)
ACUTE DIC
1442(2)
DIC in Septic Shock
1442(1)
Traumatic Shock. Head Injuries
1443(1)
Obstetric Trauma
1443(1)
Abruptio Placentae
1444(1)
NEOPLASIA AND DIC
1444(1)
APL and DIC
1445(1)
Liver Disease
1445(1)
CHRONIC DIC
1445(1)
THERAPY OF DIC: A REPRISE
1445(1)
Primary Fibrinolysis
1445(6)
MOST CASES OF "PRIMARY FIBRINOLYSIS" ARE SECONDARY TO DIC
1445(1)
USE OF FIBRINOLYSIS INHIBITORS REPRESENTS A DANGEROUS GAMBLE
1445(1)
TREATMENT
1446(1)
EACA
1446(1)
Tranexamic Acid
1446(1)
ENVENOMATION BY SNAKES
1446(1)
Pathogenesis of Venom-Induced Hemorrhagic Disorders
1447(14)
Index 1461

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