Wintrobe’s Clinical Hematology 13th Edition PDF Free Download

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Wintrobe’s Clinical Hematology 13th Edition eBook Details

  • by John P. Greer, Daniel A. Arber, Bertil Glader, Alan F. List, Robert T. Means, Frixos Paraskevas, George M. Rodgers
  • Wolters Kluwer Health
  • Release Date: October 17, 2013
  • ISBN: 9781469838205
  • Language: English
  • Edition: 13th
  • Pages: 2312
  • Copyright year: 2014
  • Format: PDF

Wintrobe’s Clinical Hematology 13th Edition PDF Book Synopsis

With the 13th edition, Wintrobe’s Clinical Hematology once again bridges the gap between the clinical practice of hematology and the basic foundations of science. Broken down into eight parts, this book provides readers with a comprehensive overview of: Laboratory Hematology, The Normal Hematologic System, Transfusion Medicine, Disorders of Red Cells, Hemostasis and Coagulation; Benign Disorders of Leukocytes, The Spleen and/or Immunoglobulins; Hematologic Malignancies, and Transplantation. Within these sections, there is a heavy focus on the morphological exam of the peripheral blood smear, bone marrow, lymph nodes, and other tissues. With the knowledge about gene therapy and immunotherapy expanding, new, up-to-date information about the process and application of these therapies is included. Likewise, the editors have completely revised material on stem cell transplantation in regards to both malignant and benign disorders, graft versus host disease, and the importance of long-term follow-up of transplantation survivors.

Related Book: Wintrobe’s Clinical Hematology 14th Edition PDF

Table Of Contents

COPYRIGHT
DEDICATION
CONTRIBUTORS
PREFACE
THE WINTROBE LEGACY
IN MEMORY OF JOHN N. LUKENS
THIRTEENTH EDITION
REFERENCES
ACKNOWLEDGMENTS

CONTENTS

INTRODUCTION
THE FOUNDATIONS OF DIAGNOSIS
PRINCIPLES OF MANAGEMENT
REFERENCES

PART I: Laboratory Hematology

CHAPTER 1: EXAMINATION OF THE BLOOD AND BONE MARROW

SPECIMEN COLLECTION
RELIABILITY OF TESTS
CELL COUNTS
RED BLOOD CELL ANALYTIC PARAMETERS
LEUKOCYTE ANALYSIS
PLATELET ANALYSIS
ADVANTAGES AND SOURCES OF ERROR WITH AUTOMATED HEMATOLOGY
MORPHOLOGIC ANALYSIS OF BLOOD CELLS
BONE MARROW EXAMINATION
SPECIAL STAINS
OTHER LABORATORY STUDIES
SELECTED REFERENCES

CHAPTER 2: CLINICAL FLOW CYTOMETRY

DEFINITION
HISTORICAL BACKGROUND
PRINCIPLES OF FLOW CYTOMETRY
NORMAL HEMATOPOIESIS
MULTICOLOR ANALYSIS OF HEMATOLOGIC MALIGNANCIES
OTHER APPLICATIONS OF FLOW CYTOMETRY IN HEMATOLOGY
STEM CELL TRANSPLANTATION
SOME APPLICATIONS OF FLOW CYTOMETRY IN IMMUNODEFICIENCY, AUTOIMMUNE, AND INFECTIOUS DISEASES
CELLULAR DNA CONTENT AND CELL CYCLE ANALYSIS
FUNCTIONAL ASSAYS
SELECTED REFERENCES

CHAPTER 3: CYTOGENETICS

INTRODUCTION
HISTORY
CYTOGENETIC ANALYSIS OF HEMATOLOGIC MALIGNANCIES
CHROMOSOME ANALYSIS
CYTOGENETIC NOMENCLATURE
FLUORESCENCE IN SITU HYBRIDIZATION ANALYSIS
FLUORESCENCE IN SITU HYBRIDIZATION NOMENCLATURE
DIAGNOSTIC AND PROGNOSTIC IMPACT OF CHROMOSOME ABNORMALITIES
ARRAY ANALYSIS
SELECTED REFERENCES

CHAPTER 4: MOLECULAR DIAGNOSIS IN HEMATOLOGY

INTRODUCTION
AN OVERVIEW OF MOLECULAR BIOLOGY
MOLECULAR DIAGNOSTIC APPLICATIONS IN HEMATOLOGY
MOLECULAR DIAGNOSIS IN THE NEXT 5 YEARS: THE COMING ASSAYS AND METHODOLOGIES
SELECTED REFERENCES
PART II: The Normal Hematologic Sysytem
SECTION 1: HEMATOPOIESIS

CHAPTER 5: ORIGIN AND DEVELOPMENT OF BLOOD CELLS

BLOOD CELLS
ORIGIN OF HEMATOPOIESIS
HEMATOPOIETIC STEM CELLS
HEMATOPOIETIC PROGENITOR CELLS
TRAFFICKING OF HEMATOPOIETIC STEM AND PROGENITOR CELLS
LINEAGE COMMITMENT
HEMATOPOIETIC MICROENVIRONMENTS
PERSPECTIVES
ACKNOWLEDGMENTS
SELECTED REFERENCES

SECTION 2: THE ERYTHROCYTE

CHAPTER 6: THE BIRTH, LIFE, AND DEATH OF RED BLOOD CELLS: ERYTHROPOIESIS, THE MATURE RED BLOOD CELL,

ERYTHROPOIESIS
ERYTHROID CELLS
BIOSYNTHESIS OF HEMOGLOBIN
CONTROL OF ERYTHROPOIESIS
ASSAYS FOR ERYTHROPOIETIN AND LEVELS IN HEALTH AND DISEASE
THE MATURE RED BLOOD CELL
STRUCTURAL FEATURES OF ERYTHROCYTES
THE ERYTHROCYTE MEMBRANE AND CYTOSKELETON
HEMOGLOBIN AND ERYTHROCYTE FUNCTION
CELL DESTRUCTION
FATE OF INTRAVASCULAR HEMOGLOBIN
EXTRAVASCULAR HEMOGLOBIN DEGRADATION
SELECTED REFERENCES

SECTION 3: GRANULOCYTES AND MONOCYTES

CHAPTER 7: NEUTROPHILIC LEUKOCYTES

SUBCELLULAR STRUCTURE OF NEUTROPHILS
MORPHOLOGY OF NEUTROPHILS AND PRECURSORS
DEVELOPMENT OF NEUTROPHILS AND THEIR PRECURSORS
MACROPOLYCYTES
GENETIC SEX AS INDICATED BY LEUKOCYTES
DIFFERENTIAL CELL COUNTING AND NORMAL VALUES FOR LEUKOCYTES
NEUTROPHIL KINETICS
PHYSIOLOGIC VARIATION IN LEUKOCYTES
CONTROL MECHANISMS REGULATING NEUTROPHIL PRODUCTION
NEUTROPHIL FUNCTION
SELECTED REFERENCES

CHAPTER 8: THE HUMAN EOSINOPHIL

INTRODUCTION
EOSINOPHIL MEDIATORS
EOSINOPHIL DIFFERENTIATION
EOSINOPHIL HETEROGENEITY
EOSINOPHIL TISSUE ACCUMULATION
EOSINOPHILS AND ALLERGIC DISEASE
THE EFFECTOR ROLE OF THE EOSINOPHIL IN WORM INFECTIONS
CONCLUSIONS
SELECTED REFERENCES
WEBSITES

CHAPTER 9: MAST CELLS AND BASOPHILS: ONTOGENY, CHARACTERISTICS, AND FUNCTIONAL DIVERSITY

INTRODUCTION
GENERAL MORPHOLOGY, MEDIATOR SECRETION, AND RECOVERY
ONTOGENY AND DEVELOPMENTAL BIOLOGY OF MAST CELLS AND BASOPHILS
CHARACTERISTICS OF MAST CELLS AND BASOPHILS
BASOPHIL AND MAST CELL FUNCTIONS
SELECTED REFERENCES

CHAPTER 10: MONOCYTES, MACROPHAGES, AND DENDRITIC CELLS

INTRODUCTION
FUNCTIONAL PROPERTIES
DISTRIBUTION OF MONONUCLEAR PHAGOCYTES
DEVELOPMENT AND RENEWAL
MONONUCLEAR PHAGOCYTES IN MEDICINE
CONCLUSION
ACKNOWLEDGMENTS
SELECTED REFERENCES

SECTION 4: THE LYMPHOCYTES

CHAPTER 11: LYMPHOCYTES AND LYMPHATIC ORGANS

LYMPHOCYTES: HISTORICAL PERSPECTIVE
LYMPHOCYTES
PLASMA CELLS
PRIMARY LYMPHOID ORGANS
SECONDARY LYMPHOID ORGANS
LYMPHOCYTE HOMING AND RECIRCULATION
SELECTED REFERENCES

CHAPTER 12: B LYMPHOCYTES

ONTOGENY
STEM CELL TO PROGENITOR B CELL
CELL INTERACTIONS IN EARLY B-CELL DEVELOPMENT
PRO-B CELL TO IG+ B CELL
IMMUNOGLOBULIN GENES
VH–D–JH GENES
V (D) J RECOMBINATION: FORMATION OF THE CODING AND SIGNAL JOINTS
DNA REPAIR MECHANISMS
RAG1 AND RAG2 PROTEINS
SOMATIC HYPERMUTATION
HUMAN HEAVY- AND LIGHT-CHAIN GENE LOCI
REGULATION OF V(D)J RECOMBINATION
BCR V GENE REPERTOIRE
THE MUTATION IN SEVERE COMBINED IMMUNODEFICIENCY
V(D)J RECOMBINATION: DNA CLEAVAGE
OMENN SYNDROME
V(D)J RECOMBINATION: DNA REPAIR
ATAXIA TELANGIECTASIA
NIJMEGEN BREAKAGE SYNDROME
BLOOM SYNDROME
DEFECTS OF LIGASES
XERODERMA PIGMENTOSUM
GENETIC DEFECTS OF B-CELL DEVELOPMENT
BRUTON AGAMMAGLOBULINEMIA
EARLY STAGES OF B-CELL DEVELOPMENT
MATURE B LYMPHOCYTES
SURFACE IMMUNOGLOBULIN
BCR COMPLEX: STRUCTURE AND SIGNALING
B-CELL RECEPTOR AND LIPID RAFTS
B-CELL RECEPTOR SIGNALING
INITIATION PATHWAY
GENERATION OF PHOSPHOINOSITIDES
THE RAS PATHWAY
ADAPTER MOLECULES: PLASTICITY AND DIVERSITY OF SIGNALING
B-CELL SIGNALING BY ACCESSORY STRUCTURES
INTERLEUKIN-4
INTERLEUKIN-5
INTERLEUKIN-6
CD 40
POLYCLONAL ACTIVATION
MORPHOLOGIC CHANGES ASOCIATED WITH B-CELL DIFFERENTIATION
MOLECULAR AND ULTRASTRUCTURAL ASPECTS OF IMMUNOGLOBULIN BIOSYNTHESIS
B-CELL ACTIVATION AND DIFFERENTIATION
B-CELL MALIGNANCIES
B-CELL SUBPOPULATIONS
SELECTED REFERENCES

CHAPTER 13: T LYMPHOCYTES AND NATURAL KILLER CELLS

MORPHOLOGY
ONTOGENY OF THYMIC MICROENVIRONMENT
T-CELL PROGENITORS
NOTCH AND T-CELL COMMITMENT
PHENOTYPIC DIFFERENTIATION
T-CELL RECEPTOR GENES
PRE-T–CELL RECEPTOR AND T-CELL-αβ, T-CELL-γδ LINEAGES
CD4/CD8 LINEAGE COMMITMENT
POSITIVE AND NEGATIVE SELECTION
THE CELLS AND MOLECULES IN THE SELECTION OF THYMOCYTES
THE MAJOR HISTOCOMPATIBILITY COMPLEX–PEPTIDE COMPLEX
PEPTIDE–MAJOR HISTOCOMPATIBILITY COMPLEX INTERACTION: THE CORECEPTORS
THE T-CELL RECEPTOR: SIGNAL TRANSDUCTION COMPONENT
CORECEPTORS TO T-CELL RECEPTOR: CD4 AND C-8 CD4
CD8
T-CELL RECEPTOR AND T-CELL ACTIVATION
CD4 T-CELL DIFFERENTIATION
CD8 T-CELL DIFFERENTIATION
GENOMIC VIEW OF TYPE 1 AND TYPE 2 DIFFERENTIATION
THE TH1—TH2 PARADIGM
CD8+ T LYMPHOCYTES
GRANULE CONTENTS
TARGET CELL LYSIS
THE FUNCTION OF CYTOTOXIC T LYMPHOCYTES
γδ T-CELLS
NATURAL KILLER CELLS: MORPHOLOGY, CYTOCHEMISTRY, AND SURFACE MARKERS
NATURAL KILLER T-CELLS
SELECTED REFERENCES

CHAPTER 14: EFFECTOR MECHANISMS IN IMMUNITY

MAJOR HISTOCOMPATIBILITY COMPLEX
MAJOR HISTOCOMPATIBILITY COMPLEX MOLECULES: NONCLASSICAL
ORIGINS OF THE MAJOR HISTOCOMPATIBILITY COMPLEX
ANTIGEN PROCESING AND PRESENTATION
DENDRITIC CELLS
T–B-CELL INTERACTIONS
GERMINAL CENTER REACTION
IMMUNOGLOBULIN CLASS SWITCH
SOMATIC HYPERMUTATION
GENOMIC VIEW OF GERMINAL CENTER REACTION
POST-GERMINAL CENTER DIFFERENTIATION
GENETIC DEFECTS OF CLASS-SWITCH RECOMBINATION
CYTOKINES IN B-CELL DIFFERENTIATION
IMMUNOGLOBULIN STRUCTURE
IMMUNOGLOBULIN FUNCTION
COMPLEMENT
REGULATION OF COMPLEMENT ACTIVATION
ANAPHYLATOXINS
COMPLEMENT COMPONENT DEFICIENCIES
THE COMPLEMENT SYSTEM
SELECTED REFERENCES

SECTION 5: HEMOSTASIS

CHAPTER 15: MEGAKARYOCYTES

HISTORICAL PERSPECTIVE
MEGAKARYOPOIESIS AND THE HEMATOPOIETIC STEM CELL
MEGAKARYOCYTE DEVELOPMENT
TRANSCRIPTIONAL REGULATION OF MEGAKARYOPOIESIS
CYTOKINES IN MEGAKARYOPOIESIS
SIGNAL TRANSDUCTION PATHWAYS IN MEGAKARYOCYTES
CLINICAL DISORDERS OF MEGAKARYOPOIESIS
SELECTED REFERENCES

CHAPTER 16: PLATELET STRUCTURE AND FUNCTION IN HEMOSTASIS AND THROMBOSIS

PLATELET STRUCTURE
PLATELET ADHESION AND ACTIVATION
PLATELET AGGREGATION:αIIBβ3 RECEPTOR AND ITS SIGNALING MECHANISMS
ROLE OF THE CYTOSKELETON IN PLATELET FUNCTION
PLATELET–CELL INTERACTIONS
ROLE OF PLATELETS IN SYSTEMIC DISORDERS
SELECTED REFERENCES

CHAPTER 17: PLATELET FUNCTION IN HEMOSTASIS AND THROMBOSIS

PLATELET ADHESION AND ACTIVATION
PLATELET AGGREGATION: αIIBβ3 RECEPTOR AND ITS SIGNALING MECHANISMS
ROLE OF THE CYTOSKELETON IN PLATELET FUNCTION
PLATELET–CELL INTERACTIONS
ROLE OF PLATELETS IN ATHEROGENESIS AND ATHEROTHROMBOSIS
ACKNOWLEDGMENTS
SELECTED REFERENCES

CHAPTER 18: BLOOD COAGULATION AND FIBRINOLYSIS

ESSENTIAL FEATURES OF COAGULATION
OVERVIEW OF PROCOAGULANT PATHWAYS: PRIMARY (EXTRINSIC) AND ACCESSORY (INTRINSIC) PATHWAYS
PROCOAGULANT PROTEINS: ACCESSORY PATHWAY FACTORS AND FACTOR XI
PROCOAGULANT PROTEINS: VITAMIN K–DEPENDENT PROTEIN FAMILY
PROCOAGULANT PROTEINS: PROCOAGULANT COFACTOR PROTEINS
PROCOAGULANT PROTEINS: THROMBIN (FACTOR IIa)
ANTICOAGULANT PROTEINS: DYNAMIC INHIBITORY SYSTEM
ANTICOAGULANT PROTEINS: PROTEINASE INHIBITORS
ANTICOAGULANT PROTEINS: SERINE PROTEASE INHIBITOR SUPERFAMILY (SERPIN)
PROTEINS OF CLOT FORMATION
PROTEINS OF THE FIBRINOLYTIC SYSTEM
INHIBITORS OF THE FIBRINOLYTIC SYSTEM
PHYSIOLOGIC REGULATION OF FIBRINOLYSIS
CONCLUSION
ACKNOWLEDGMENTS
SELECTED REFERENCES

CHAPTER 19: ENDOTHELIUM: ANGIOGENESIS AND THE REGULATION OF HEMOSTASIS

NORMAL ANGIOGENESIS
ENDOTHELIAL CELL STRUCTURE
THE VESSEL WALL AND HEMOSTASIS: GENERAL CONCEPTS
ANTITHROMBOTIC PROPERTIES OF UNPERTURBED ENDOTHELIUM
PROTHROMBOTIC PROPERTIES OF UNPERTURBED ENDOTHELIUM
HEMOSTATIC PROPERTIES OF PERTURBED ENDOTHELIUM
SELECTED REFERENCES

PART III: Transfusion Medicine

CHAPTER 20: RED CELL, PLATELET, AND WHITE CELL ANTIGENS

INTRODUCTION
RED BLOOD CELL ANTIGENS
PLATELET ANTIGENS
WHITE BLOOD CELL ANTIGENS
SELECTED REFERENCES

CHAPTER 21: TRANSFUSION MEDICINE

BLOOD DONATION AND COLLECTION
BLOOD COLLECTION PROCESS
RED BLOOD CELL PRESERVATION AND STORAGE
PLATELET PREPARATION AND STORAGE
PREPARATION OF PLASMA COMPONENTS
BLOOD COMPONENT MODIFICATION
ALTERNATIVES TO ALLOGENEIC DONOR BLOOD
USE OF BLOOD COMPONENTS
RED CELL TRANSFUSION
PLATELET TRANSFUSION
PLASMA TRANSFUSION
GRANULOCYTE TRANSFUSIONS
ADVERSE EFFECTS OF BLOOD TRANSFUSION
INFECTIOUS COMPLICATIONS OF BLOOD TRANSFUSION
THERAPEUTIC APHERESIS
SUMMARY
SELECTED REFERENCES

PART IV: Disorders of Red Cells

SECTION 1: INTRODUCTION

CHAPTER 22: ANEMIA: GENERAL CONSIDERATIONS

DEFINITION OF ANEMIA
CLINICAL EFFECTS OF ANEMIA
EVALUATION AND CLASSIFICATION OF ANEMIA
APPROACH TO MACROCYTIC ANEMIA
APPROACH TO MICROCYTIC ANEMIA
GENERAL DIAGNOSTIC APPROACH TO HEMOGLOBINOPATHIES AND THALASSEMIAS
APPROACH TO NORMOCYTIC ANEMIA
APPROACH TO HEMOLYSIS
APPROACH TO ACUTE POSTHEMORRHAGIC ANEMIA
SELECTED REFERENCES

SECTION 2: DISORDERS OF IRON METABOLISM AND HEME SYNTHESIS

CHAPTER 23: IRON DEFICIENCY AND RELATED DISORDERS

NORMAL IRON PHYSIOLOGY
IRON DEFICIENCY ANEMIA
CLINICAL FEATURES OF IRON DEFICIENCY
LABORATORY EVALUATION
MANAGEMENT OF IRON DEFICIENCY
ACKNOWLEDGMENTS
SELECTED REFERENCES

CHAPTER 24: SIDEROBLASTIC ANEMIAS

HISTORICAL ASPECTS
HEME SYNTHESIS IN ERYTHROID CELLS
CONGENITAL SIDEROBLASTIC ANEMIAS
ACQUIRED CLONAL SIDEROBLASTIC ANEMIA
REVERSIBLE SIDEROBLASTIC ANEMIAS
ANEMIA FROM DEFECTS IN ERYTHROID HEME SYNTHESIS WITHOUT RING SIDEROBLASTS
KEY CLINICAL SUMMARY POINTS
WEBSITE RESOURCE
SELECTED REFERENCES

CHAPTER 25: HEMOCHROMATOSIS

IRON OVERLOAD
HEMOCHROMATOSIS
HISTORY
ETIOLOGY
PATHOPHYSIOLOGY
ORGAN AND CELLULAR INJURY DUE TO IRON OVERLOAD
DIFFERENTIAL DIAGNOSIS OF IRON OVERLOAD
HFE HEMOCHROMATOSIS GENE
FREQUENCY OF HEMOCHROMATOSIS IN MEN AND WOMEN
CLINICAL FEATURES OF HEMOCHROMATOSIS
HFE MUTATIONS AND OTHER CONDITIONS
DIAGNOSIS
MANAGEMENT
PROGNOSIS
IMAGING OF THE LIVER
SELECTED REFERENCES

CHAPTER 26: PORPHYRIAS

PORPHYRIAS WITH CUTANEOUS PHOTOSENSITIVITY
ACUTE OR INDUCIBLE PORPHYRIAS WITH NEUROLOGIC MANIFESTATIONS
ACUTE OR INDUCIBLE PORPHYRIAS WITH BOTH NEUROLOGIC AND CUTANEOUS MANIFESTATIONS
DUAL PORPHYRIA
KEY CLINICAL SUMMARY POINTS
WEBSITE RESOURCES
SELECTED REFERENCES

SECTION 3: HEMOLYTIC ANEMIA

CHAPTER 27: HEREDITARY SPHEROCYTOSIS, HEREDITARY ELLIPTOCYTOSIS, AND OTHER DISORDERS ASSOCIATED WITH

THE ERYTHROCYTE MEMBRANE
HEREDITARY SPHEROCYTOSIS
HEREDITARY ELLIPTOCYTOSIS SYNDROMES
STOMATOCYTIC DISORDERS
ACANTHOCYTIC DISORDERS
ECHINOCYTIC DISORDERS
TARGET CELL DISORDERS
SELECTED REFERENCES
WEBSITE RESOURCES

CHAPTER 28: HEREDITARY HEMOLYTIC ANEMIAS DUE TO RED BLOOD CELL ENZYME DISORDERS

OVERVIEW OF ERYTHROCYTE METABOLISM
DISORDERS OF HEXOSE MONOPHOSPHATE SHUNT AND GLUTATHIONE METABOLISM
GLYCOLYTIC ENZYME ABNORMALITIES—GENERAL CONSIDERATIONS
ABNORMALITIES OF PURINE AND PYRIMIDINE NUCLEOTIDE METABOLISM
SELECTED REFERENCES

CHAPTER 29: AUTOIMMUNE HEMOLYTIC ANEMIA

CLASSIFICATION
IMMUNE HEMOLYTIC ANEMIAS CAUSED BY COLD-ACTIVE ANTIBODIES
IMMUNE HEMOLYTIC ANEMIAS CAUSED BY MIXED COLD- AND WARM-ACTIVE ANTIBODIES
IMMUNE HEMOLYTIC ANEMIAS CAUSED BY WARM-ACTIVE ANTIBODIES
DRUG-INDUCED IMMUNE HEMOLYTIC ANEMIA
TRANSPLANT-ASSOCIATED IMMUNE HEMOLYTIC ANEMIAS
SELECTED REFERENCES

CHAPTER 30: HEMOLYTIC DISEASE OF THE FETUS AND NEWBORN

HISTORICAL BACKGROUND
PATHOPHYSIOLOGY
CLINICAL FEATURES
LABORATORY EVALUATION
PREVENTION OF MATERNAL D ALLOIMMUNIZATION WITH RH IMMUNE GLOBULIN
MANAGEMENT OF RED BLOOD CELL ALLOIMMUNIZATION IN PREGNANCY
TREATMENT OF THE NEWBORN INFANT
CLINICAL OUTCOMES OF TREATMENT FOR HEMOLYTIC DISEASE OF THE FETUS AND NEWBORN
CONCLUSION
SELECTED REFERENCES

CHAPTER 31: PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

INTRODUCTION
ETIOLOGY AND PATHOGENESIS
CLINICAL MANIFESTATIONS
LABORATORY FINDINGS
DIFFERENTIAL DIAGNOSIS
TREATMENT
DISEASE COURSE AND PROGNOSIS
SELECTED REFERENCES

CHAPTER 32: ACQUIRED NONIMMUNE HEMOLYTIC DISORDERS

HEMOLYSIS DUE TO INFECTION
HEMOLYSIS DUE TO DRUGS AND CHEMICALS
HEMOLYSIS WITH VENOMS
HEMOLYSIS WITH THERMAL INJURY
FRAGMENTATION HEMOLYSIS
OTHER CAUSES OF HEMOLYSIS
POSTPERFUSION SYNDROME
SELECTED REFERENCES

SECTION 4: HEREDITARY DISORDERS OF HEMOGLOBIN STRUCTURE AND SYNTHESIS

CHAPTER 33: SICKLE CELL ANEMIA AND OTHER SICKLING SYNDROMES

EPIDEMIOLOGY
PATHOPHYSIOLOGY
SICKLE CELL ANEMIA (HEMOGLOBIN SS)
SICKLE CELL TRAIT
OTHER SICKLING SYNDROMES
KEY POINTS
WEBSITES OF INTEREST
SELECTED REFERENCES

CHAPTER 34: THALASSEMIAS AND RELATED DISORDERS: QUANTITATIVE DISORDERS OF HEMOGLOBIN SYNTHESIS

PREVALENCE, GEOGRAPHIC DISTRIBUTION, AND THE ROLE OF MALARIA
GENETIC MECHANISMS AND MOLECULAR PATHOLOGY
PATHOPHYSIOLOGY
GENOTYPE–PHENOTYPE CORRELATION IN THALASSEMIA
CLINICAL AND LABORATORY FEATURES
β-THALASSEMIA MAJOR
THALASSEMIA MINOR
THALASSEMIA INTERMEDIA
β-THALASSEMIA IN ASSOCIATION WITH β-CHAIN STRUCTURAL VARIANTS
WEBSITE
SELECTED REFERENCES

CHAPTER 35: HEMOGLOBINS WITH ALTERED OXYGEN AFFINITY, UNSTABLE HEMOGLOBINS, M-HEMOGLOBINS, AND DYSHE

HEMOGLOBINS WITH ALTERED OXYGEN AFFINITY
UNSTABLE HEMOGLOBINS
M HEMOGLOBINS
METHEMOGLOBINEMIA UNRELATED TO GLOBIN GENE MUTATIONS
PULSE OXIMETRY
DYSHEMOGLOBINEMIAS: CARBOXY-, SULF-, AND NITROSOHEMOGLOBINS
SELECTED REFERENCES

SECTION 5: OTHER RED CELL DISORDERS

CHAPTER 36: MEGALOBLASTIC ANEMIAS: DISORDERS OF IMPAIRED DNA SYNTHESIS

HISTORICAL BACKGROUND
NORMAL PHYSIOLOGY AND PATHOPHYSIOLOGY
CLINICAL AND LABORATORY FEATURES
SPECIFIC DISORDERS CAUSING COBALAMIN OR FOLATE DEFICIENCY
MANAGEMENT
SELECTED REFERENCES

CHAPTER 37: INHERITED APLASTIC ANEMIA SYNDROMES

FANCONI ANEMIA
DYSKERATOSIS CONGENITA
SHWACHMAN-DIAMOND SYNDROME
CONGENITAL AMEGAKARYOCYTIC THROMBOCYTOPENIA
SELECTED REFERENCES

CHAPTER 38: ACQUIRED APLASTIC ANEMIA

HISTORICAL BACKGROUND
ACQUIRED VERSUS CONSTITUTIONAL APLASTIC ANEMIA
EPIDEMIOLOGY
PATHOPHYSIOLOGY
CLINICAL FEATURES AND DIAGNOSIS
SUPPORTIVE CARE
DEFINITIVE TREATMENT
SELECTED REFERENCES

CHAPTER 39: RED CELL APLASIA: ACQUIRED AND CONGENITAL DISORDERS

ACQUIRED RED CELL APLASIA
DIAMOND-BLACKFAN ANEMIA
SELECTED REFERENCES

CHAPTER 40: CONGENITAL DYSERYTHROPOIETIC ANEMIAS

CONGENITAL DYSERYTHROPOIETIC ANEMIA TYPE I (CDA I)
CONGENITAL DYSERYTHROPOIETIC ANEMIA TYPE II (CDA II)
CONGENITAL DYSERYTHROPOIETIC ANEMIA TYPE III (CDA III)
CONGENITAL DYSERYTHROPOIETIC ANEMIA VARIANTS
SELECTED REFERENCES

CHAPTER 41: ANEMIAS SECONDARY TO CHRONIC DISEASE AND SYSTEMIC DISORDERS

ANEMIA OF CHRONIC DISEASE
ANEMIA OF CHRONIC RENAL INSUFFICIENCY
ANEMIA IN CIRRHOSIS AND OTHER LIVER DISEASES
ANEMIAS ASSOCIATED WITH ENDOCRINE DISORDERS
SELECTED REFERENCES

CHAPTER 42: ANEMIAS DURING PREGNANCY AND THE POSTPARTUM PERIOD

OVERVIEW/EPIDEMIOLOGY
MATERNAL ERYTHROPOIESIS
PHYSIOLOGIC ANEMIA OF PREGNANCY
IRON DEFICIENCY DURING PREGNANCY
DEFICIENCY OF FOLATE AND OTHER NUTRIENTS DURING PREGNANCY
MATERNAL ANEMIA ASSOCIATED WITH PRENATAL INFECTIONS
HEMOLYTIC ANEMIA IN PREGNANCY
BONE MARROW FAILURE SYNDROMES ASSOCIATED WITH PREGNANCY
POSTPARTUM ANEMIA
SELECTED REFERENCES

CHAPTER 43: ANEMIAS UNIQUE TO THE FETUS AND NEONATE

INTRODUCTION
ERYTHROPOIETIN BIOLOGY IN THE FETUS AND NEONATE
NORMAL ERYTHROCYTE VALUES DURING HUMAN FETAL DEVELOPMENT
FETAL AND NEONATAL ERYTHROCYTE MEMBRANE AND METABOLISM
FETAL AND NEONATAL ANEMIA DUE TO HEMOLYSIS
FETAL AND NEONATAL ANEMIA DUE TO HEMORRHAGE
FETAL AND NEONATAL ANEMIA DUE TO CONGENITAL INFECTION
ANEMIA OF PREMATURITY AND OTHER HYPOPROLIFERATIVE DISORDERS
CONSIDERATIONS REGARDING ERYTHROCYTE TRANSFUSION IN THE NEONATAL PERIOD
SELECTED REFERENCES

CHAPTER 44: ERYTHROCYTOSIS

DEFINITIONS AND TERMINOLOGY
PATHOLOGIC PHYSIOLOGY
CLASSIFICATION AND APPROACH TO THE PATIENT WITH ERYTHROCYTOSIS
RELATIVE POLYCYTHEMIA
POLYCYTHEMIA (ABSOLUTE ERYTHROCYTOSIS)
SELECTED REFERENCES
PART V: Disorders of Hemostasis and Coagulation
SECTION 1: INTRODUCTION

CHAPTER 45: DIAGNOSTIC APPROACH TO THE BLEEDING DISORDERS

CLINICAL EVALUATION OF THE BLEEDING PATIENT
MANIFESTATIONS OF DISORDERED HEMOSTASIS
CLINICAL FEATURES OF INHERITED BLEEDING DISORDERS
CLINICAL FEATURES OF ACQUIRED BLEEDING DISORDERS
LABORATORY METHODS FOR STUDY OF HEMOSTASIS AND BLOOD COAGULATION
INITIAL LABORATORY EVALUATION
CONFIRMATORY TESTS
PREOPERATIVE HEMOSTASIS EVALUATION
EVALUATION OF THE NEONATE
SELECTED REFERENCES

SECTION 2: THROMBOCYTOPENIA

CHAPTER 46: THROMBOCYTOPENIA: PATHOPHYSIOLOGY AND CLASSIFICATION

PATHOPHYSIOLOGY
CLASSIFICATION
SELECTED REFERENCES

CHAPTER 47: THROMBOCYTOPENIA CAUSED BY IMMUNOLOGIC PLATELET DESTRUCTION

PRIMARY IMMUNE THROMBOCYTOPENIA
SECONDARY AUTOIMMUNE THROMBOCYTOPENIC PURPURA
ALLOIMMUNE THROMBOCYTOPENIA
SELECTED REFERENCES

CHAPTER 48: THROMBOTIC THROMBOCYTOPENIC PURPURA, HEMOLYTIC-UREMIC SYNDROME, AND RELATED DISORDERS

ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA
SHIGA TOXIN–ASOCIATED HEMOLYTIC-UREMIC SYNDROME
ATYPICAL HEMOLYTIC–UREMIC SYNDROME
PLATELET AND RED CELL DAMAGE BY ABNORMAL VASCULAR SURFACES
MISCELLANEOUS FORMS OF NONIMMUNOLOGIC PLATELET DESTRUCTION
SELECTED REFERENCES

CHAPTER 49: MISCELLANEOUS CAUSES OF THROMBOCYTOPENIA

CONGENITAL THROMBOCYTOPENIA
ACQUIRED THROMBOCYTOPENIA
SELECTED REFERENCES

CHAPTER 50: BLEEDING DISORDERS CAUSED BY VASCULAR ABNORMALITIES

CLINICAL APPROACH TO THE PATIENT
MECHANICAL PURPURA
STRUCTURAL MALFORMATIONS OF VESSELS
BLEEDING DUE TO DISORDERS OF PERIVASCULAR TISSUE
VASCULITIS
PURPURA ASSOCIATED WITH INFECTION
PURPURA ASSOCIATED WITH VASCULAR OBSTRUCTION
PURPURA ASSOCIATED WITH SKIN DISEASES
PSYCHOGENIC PURPURA
SELECTED REFERENCES

CHAPTER 51: THROMBOCYTOSIS AND ESSENTIAL THROMBOCYTHEMIA

REACTIVE (SECONDARY) THROMBOCYTOSIS
DIFFERENTIAL DIAGNOSIS AND CLINICAL APPROACH TO THROMBOCYTOSIS
ESSENTIAL THROMBOCYTHEMIA
TREATMENT
SELECTED REFERENCES

CHAPTER 52: QUALITATIVE DISORDERS OF PLATELET FUNCTION

NORMAL PLATELET FUNCTION: A SUMMARY
DIAGNOSIS AND CLASSIFICATION OF PLATELET DYSFUNCTION: AN ALGORITHM
HEREDITARY DISORDERS OF PLATELET FUNCTION
ACQUIRED DISORDERS OF PLATELET FUNCTION
SELECTED REFERENCES

SECTION 4: COAGULATION DISORDERS

CHAPTER 53: INHERITED COAGULATION DISORDERS

NOMENCLATURE
PRINCIPLES OF PATHOPHYSIOLOGY
HEMOPHILIA A
HEMOPHILIA B
VON WILLEBRAND DISEASE
CLINICAL DISORDERS OF THE FIBRINOGEN MOLECULE
FACTOR XIII DEFICIENCY
PROTHROMBIN DEFICIENCY
FACTOR V DEFICIENCY
FACTOR VII DEFICIENCY
FACTOR X DEFICIENCY
FACTOR XI DEFICIENCY
FACTOR XII DEFICIENCY
PREKALLIKREIN DEFICIENCY
HIGH-MOLECULAR-WEIGHT KININOGEN DEFICIENCY
MISCELLANEOUS INHERITED COAGULATION DISORDERS
ABNORMALITIES OF PROTEASE INHIBITORS
TREATMENT OF INHERITED COAGULATION DISORDERS
MAJOR AND MINOR BLEEDING
SPECIAL ASPECTS OF TREATMENT
THE FUTURE IN HEMOPHILIA TREATMENT
SELECTED REFERENCES

CHAPTER 54: ACQUIRED COAGULATION DISORDERS

DEFICIENCIES OF VITAMIN K–DEPENDENT FACTORS
LIVER DISEASE
DISSEMINATED INTRAVASCULAR COAGULATION
PRIMARY FIBRINOLYSIS (FIBRINOGENOLYSIS)
SELECTED REFERENCES

SECTION 5: THROMBOSIS

CHAPTER 55: THROMBOSIS AND ANTITHROMBOTIC THERAPY

PHYSIOLOGY AND PATHOPHYSIOLOGY OF THROMBOSIS
VIRCHOW TRIAD
ACTIVATION OF COAGULATION
INHERITED THROMBOTIC DISORDERS
ANTITHROMBOTIC THERAPY
VENOUS THROMBOEMBOLIC DISEASE
PREVENTION OF VENOUS THROMBOEMBOLIC DISEASE
HEPARIN-INDUCED THROMBOCYTOPENIA
SELECTED REFERENCES
PART VI: Benign Disorders of Leukocytes, the Spleen, and/or Immunoglobins

CHAPTER 56: DIAGNOSTIC APPROACH TO MALIGNANT AND NONMALIGNANT DISORDERS OF THE PHAGOCYTIC AND IMMUNE

APPROACH TO DIAGNOSIS
EXAMINATION OF THE BLOOD
EXAMINATION OF THE BONE MARROW
EXAMINATION OF LYMPH NODES AND TISSUES
EXAMINATION OF THE SPLEEN
FEVER OF UNKNOWN ORIGIN
RECURRENT INFECTIONS
SUMMARY
SELECTED REFERENCES

CHAPTER 57: NEUTROPENIA

INTRODUCTION
NORMAL NEUTROPHIL KINETICS
DEFINITION AND CLASSIFICATION OF NEUTROPENIA
DIFFERENTIAL DIAGNOSIS
CLINICAL PRESENTATION AND DIAGNOSTIC APPROACH TO NEUTROPENIA
MANAGEMENT OF NEUTROPENIA
SELECTED REFERENCES

CHAPTER 58: QUALITATIVE DISORDERS OF LEUKOCYTES

DISORDERS OF PHAGOCYTIC LEUKOCYTES CHARACTERIZED BY MORPHOLOGIC CHANGES
FUNCTIONAL DISORDERS OF LEUKOCYTES NOT CHARACTERIZED BY MORPHOLOGIC CHANGES
FREQUENCY OF IDENTIFIABLE QUALITATIVE NEUTROPHIL DISORDERS
WEB SITES
SELECTED REFERENCES

CHAPTER 59: LYSOSOMAL ABNORMALITIES OF THE MONOCYTE–MACROPHAGE SYSTEM: GAUCHER AND NIEMANN-PICK DI

PATHOPHYSIOLOGY OF THE LYSOSOMAL STORAGE DISEASES
GAUCHER DISEASE
NIEMANN-PICK DISEASE
WEB SITES
SELECTED REFERENCES

CHAPTER 60: LANGERHANS CELL HISTIOCYTOSIS

HISTORY
EPIDEMIOLOGY
PATHOLOGY AND PATHOPHYSIOLOGY
CLINICAL FEATURES
DIAGNOSIS
PROGNOSIS
MANAGEMENT AND TREATMENT
SELECTED REFERENCES

CHAPTER 61: PATHOLOGY OF LANGERHANS CELL HISTIOCYTOSIS AND OTHER HISTIOCYTIC PROLIFERATIONS

LANGERHANS CELL HISTIOCYTOSIS
LANGERHANS CELL SARCOMA
FOLLICULAR DENDRITIC CELL SARCOMA
INTERDIGITATING DENDRITIC CELL SARCOMA
OTHER RARE DENDRITIC CELL SARCOMAS
BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM
HISTIOCYTIC SARCOMA
DISSEMINATED JUVENILE XANTHOGRANULOMA
ROSAI-DORFMAN DISEASE
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
SELECTED REFERENCES

CHAPTER 62: INFECTIOUS MONONUCLEOSIS AND OTHER EPSTEIN-BARR VIRUS–RELATED DISORDERS

HISTORICAL BACKGROUND
PATHOPHYSIOLOGY OF EPSTEIN-BARR VIRUS INFECTION
INFECTIOUS MONONUCLEOSIS
OTHER EPSTEIN-BARR VIRUS–RELATED DISEASES
SELECTED REFERENCES

CHAPTER 63: PRIMARY IMMUNODEFICIENCY SYNDROMES

INTRODUCTION
MAJOR COMPARTMENTS OF THE IMUNE SYSTEM
COMPLEMENT
PHAGOCYTES
B CELLS/ANTIBODIES
T CELLS
OTHER COMPLEX OR COMBINED IMUNODEFICIENCY DISORDERS
BASIC LABORATORY WORK-UP FOR IMMUNE DEFICIENCY
USEFUL WEB SITES
SELECTED REFERENCES

CHAPTER 64: ACQUIRED IMMUNODEFICIENCY SYNDROME

HUMAN IMMUNODEFICIENCY VIRUS INFECTION AND ACQUIRED IMMUNODEFICIENCY SYNDROME
HEMATOLOGIC COMPLICATIONS OF HUMAN IMMUNODEFICIENCY VIRUS INFECTION
ACQUIRED IMMUNODEFICIENCY SYNDROME–RELATED MALIGNANCIES
ACQUIRED IMMUNODEFICIENCY SYNDROME–RELATED LYMPHOMA
CONCLUSION
ACKNOWLEDGMENTS
SELECTED REFERENCES

CHAPTER 65: DISORDERS OF THE SPLEEN

DEVELOPMENT AND ANATOMY
SPLENIC FUNCTION
SPLENIC DISORDERS AND INDICATIONS FOR SPLENECTOMY
SPLENECTOMY: SURGICAL TECHNIQUE
COMPLICATIONS OF SPLENECTOMY
SELECTED REFERENCES

CHAPTER 66: TUMORS OF THE SPLEEN

PATTERNS OF TUMOROUS SPLEEN PROLIFERATIONS
CYSTS AND ABSCESSES
VASCULAR PROLIFERATIONS
LYMPHOID PROLIFERATIONS
ACUTE LEUKEMIA AND MYELOPROLIFERATIVE NEOPLASMS
OTHER TUMOROUS PROLIFERATIONS
SELECTED REFERENCES

PART VII: Hematologic Malignancies

SECTION 1: GENERAL ASPECTS

CHAPTER 67: HEMATOPOIETIC NEOPLASMS: PRINCIPLES OF PATHOLOGIC DIAGNOSIS

PREOPERATIVE CONSIDERATIONS
SPECIMEN PROCESSING
SAMPLING METHODS AND THEIR LIMITATIONS
IMUNOPHENOTYPING
GENETIC TESTING
OTHER ANCILLARY METHODS
THE DIAGNOSTIC REPORT
SELECTED REFERENCES

CHAPTER 68: PRINCIPLES AND PHARMACOLOGY OF CHEMOTHERAPY

PRINCIPLES OF CHEMOTHERAPY
DRUGS USED TO TREAT CANCER
SELECTED REFERENCES

CHAPTER 69: SUPPORTIVE CARE IN HEMATOLOGIC MALIGNANCIES

INFECTIOUS DISEASE ISSUES RELATED TO HEMATOLOGIC MALIGNANCY
SPECIFIC FEBRILE SYNDROMES, SELECTED PATHOGENS, AND TREATMENTS
CENTRAL NERVOUS SYSTEM INFECTIONS
OROPHARYNGEAL AND SINUS INFECTIONS
LUNG INFECTIONS
GASTROINTESTINAL INFECTIONS
GENITOURINARY INFECTIONS
SKIN AND SOFT TISSUE INFECTIONS
VIRAL INFECTIONS
INFECTIONS IN HEMATOPOIETIC STEM CELL TRANSPLANTATION PATIENTS
STRATEGIES TO PREVENT INFECTIONS
MANAGEMENT OF CATHETER-RELATED INFECTIONS
CHEMOTHERAPY-INDUCED NAUSEA AND VOMITING
CANCER-RELATED FATIGUE
CANCER PAIN
MUCOSITIS
ANOREXIA
COMPLICATIONS OF HEMATOLOGIC MALIGNANCIES
SUMMARY
SELECTED REFERENCES

CHAPTER 70: IMMUNOTHERAPY

HISTORY
INNATE IMMUNITY AGAINST TUMORS
ADAPTIVE IMMUNITY AGAINST TUMORS
TUMOR-ASOCIATED ANTIGENS
TUMOR-HOST INTERACTIONS: BEYOND IMMUNOSURVEILLANCE
IMMUNE EVASION BY TUMORS
APPROACHES TO IMMUNOTHERAPY
FUTURE DIRECTIONS
WEB SITES
ACKNOWLEDGEMENTS
SELECTED REFERENCES

CHAPTER 71: GENE THERAPY FOR HEMATOLOGIC DISORDERS

INTRODUCTION
OVERVIEW OF BASIC CONCEPTS
HEMATOPOIETIC STEM AND PROGENITOR CELLS AS TARGETS FOR GENE TRANSFER
LYMPHOCYTES AS TARGETS FOR GENE TRANSFER
GENE THERAPY FOR BLEEDING DISORDERS
SAFETY AND FEASIBILITY HURDLES TO CLINICAL GENE THERAPY
CONCLUSIONS
SELECTED REFERENCES
SECTION 2: THE ACUTE LEUKEMIAS

CHAPTER 72: MOLECULAR GENETICS OF ACUTE LEUKEMIA

INTRODUCTION
ACUTE MYELOID LEUKEMIA
B-ACUTE LYMPHOBLASTIC LEUKEMIA
T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA/LYMPHOMA
SUMMARY
SELECTED REFERENCES

CHAPTER 73: DIAGNOSIS AND CLASSIFICATION OF THE ACUTE LEUKEMIAS AND MYELODYSPLASTIC SYNDROMES

INTRODUCTION
DIAGNOSTIC EVALUATION
MYELODYSPLASTIC SYNDROMES
ACUTE MYELOID LEUKEMIA
ACUTE LYMPHOBLASTIC LEUKEMIA
ACUTE LEUKEMIAS OF AMBIGUOUS LINEAGE
SUMMARY
SELECTED REFERENCES

CHAPTER 74: ACUTE LYMPHOBLASTIC LEUKEMIA IN ADULTS

HISTORICAL BACKGROUND
PATHOPHYSIOLOGY
CLINICAL FEATURES
LABORATORY FEATURES
DIFFERENTIAL DIAGNOSIS
PROGNOSTIC FACTORS
PRIMARY THERAPY
SALVAGE THERAPY
SUMMARY AND FUTURE DIRECTIONS
SELECTED REFERENCES

CHAPTER 75: ACUTE MYELOID LEUKEMIA IN ADULTS

HISTORICAL PERSPECTIVE
EPIDEMIOLOGY
RISK FACTORS
CLINICAL PRESENTATION
LABORATORY FINDINGS
BIOLOGIC FEATURES
CYTOGENETIC AND MOLECULAR FINDINGS
CLASSIFICATION OF ACUTE MYELOID LEUKEMIA
CLINICOPATHOLOGIC SYNDROMES
PROGNOSIS
THERAPY
STEM CELL TRANSPLANTATION
FUTURE DIRECTIONS
SELECTED REFERENCES

CHAPTER 76: ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN

PRESENTING CLINICAL FEATURES
PRESENTING LABORATORY AND RADIOGRAPHIC FEATURES
RISK STRATIFICATION
TREATMENT
OUTCOMES
SUMMARY
SELECTED REFERENCES

CHAPTER 77: ACUTE MYELOGENOUS LEUKEMIA IN CHILDREN

HISTORICAL BACKGROUND
EPIDEMIOLOGY
CELLULAR AND MOLECULAR ORIGINS OF ACUTE MYELOGENOUS LEUKEMIA: HEMATOPOIETIC HIERARCHIES
PREDISPOSING FACTORS AND PATHOPHYSIOLOGY
PRESENTATION
DIFFERENTIAL DIAGNOSIS
ACUTE MYELOGENOUS LEUKEMIA SUBTYPES
THERAPY FOR PATIENTS WITH NEWLY DIAGNOSED ACUTE MYELOGENOUS LEUKEMIA
THERAPY FOR PATIENTS WITH RELAPSED/REFRACTORY DISEASE
SUPPORTIVE CARE
LATE EFFECTS OF THERAPY
MANAGEMENT OF PATIENTS WITH DOWN SYNDROME
MANAGEMENT OF PATIENTS WITH NON-DOWN SYNDROME INHERITED SYNDROMES
MANAGEMENT OF INFANTS WITH ACUTE MYELOGENOUS LEUKEMIA
MANAGEMENT OF ACUTE PROMYELOCYTIC LEUKEMIA
GRANULOCYTIC SARCOMA
BIPHENOTYPIC LEUKEMIA
SECONDARY OR THERAPY-RELATED ACUTE MYELOGENOUS LEUKEMIA
PROGNOSTIC FACTORS
FUTURE THERAPEUTIC CHALLENGES
SELECTED REFERENCES

CHAPTER 78: ACUTE PROMYELOCYTIC LEUKEMIA

EPIDEMIOLOGY
CLINICAL PRESENTATION
LABORATORY ANALYSIS
THERAPY OF ACUTE PROMYELOCYTIC LEUKEMIA
SUMMARY
SELECTED REFERENCES

CHAPTER 79: THE MYELODYSPLASTIC SYNDROMES

ABSTRACT
INTRODUCTION
EPIDEMIOLOGY AND ETIOLOGY
THERAPEUTIC APPROACHES FOR PATIENTS WITH MYELODYSPLASTIC SYNDROME
CONCLUSION
SELECTED REFERENCES
SECTION 3: MYELOPROLIFERATIVE DISORDERS

CHAPTER 80: PATHOLOGY OF THE MYELOPROLIFERATIVE NEOPLASMS

MYELOPROLIFERATIVE NEOPLASMS
MYELOID AND LYMPHOID NEOPLASMS WITH EOSINOPHILIA AND ABNORMALITIES OF PDGFRA, PDGFRB, OR FGFR1
MYELODYSPLASTIC/MYELOPROLIFERATIVE NEOPLASMS
CONCLUSION
SELECTED REFERENCES

CHAPTER 81: CHRONIC MYELOID LEUKEMIA

HISTORICAL PERSPECTIVE
PATHOPHYSIOLOGY
CLINICAL FEATURES
DIAGNOSIS AND INITIAL WORK-UP
MONITORING RESPONSE TO THERAPY
THERAPY
APPROACH TO THE NEWLY DIAGNOSED CHRONIC-PHASE PATIENT
APPROACH TO THE PATIENT PRESENTING IN ACCELERATED PHASE/BLASTIC PHASE
RESISTANCE TO TYROSINE KINASE INHIBITORS
APPROACH TO THE PATIENT WITH TYROSINE KINASE INHIBITOR RESISTANCE
DISCONTINUATION OF TYROSINE KINASE INHIBITOR THERAPY
ALLOGENEIC STEM CELL TRANSPLANTATION
FUTURE PERSPECTIVES
SELECTED REFERENCES

CHAPTER 82: POLYCYTHEMIA VERA

DEFINITION AND HISTORY
EPIDEMIOLOGY
CLINICAL FEATURES
BLOOD AND LABORATORY FINDINGS
PATHOGENESIS
DIAGNOSIS
NATURAL HISTORY
TREATMENT
SELECTED REFERENCES

CHAPTER 83: MYELOFIBROSIS

DEFINITION
HISTORICAL PERSPECTIVE
EPIDEMIOLOGY
PATHOGENESIS
CLINICAL FEATURES
DIAGNOSIS
DISEASE COURSE
RISK STRATIFICATION AND PROGNOSIS
MANAGEMENT
SUMMARY
SELECTED REFERENCES

CHAPTER 84: EOSINOPHILIC NEOPLASMS AND HYPEREOSINOPHILIC SYNDROME

HISTORICAL BACKGROUND
DEFINITION OF EOSINOPHILIA
EPIDEMIOLOGY
EOSINOPHIL PHYSIOLOGY
MODERN CLASSIFICATION
DIAGNOSTIC EVALUATION FOR HYPEREOSINOPHILIA
BIOLOGY OF FIP1L1–PDGFRA
CLINICAL PRESENTATION AND PROGNOSIS
TREATMENT
SUMMARY
SELECTED REFERENCES

CHAPTER 85: SYSTEMIC MASTOCYTOSIS

HISTORICAL BACKGROUND
PATHOPHYSIOLOGY
CLINICAL FEATURES
LABORATORY FINDINGS
DIFFERENTIAL DIAGNOSIS
MANAGEMENT
DEVELOPMENT OF CLINICAL RESPONSE CRITERIA TO ASSESS EFFICACY TO THERAPY
WEBSITES
SELECTED REFERENCES

SECTION 4: LYMPHOPROLIFERATIVE DISORDERS

CHAPTER 86: DIAGNOSIS AND CLASSIFICATION OF LYMPHOMAS

SPECIMEN EVALUATION
B-CELL LYMPHOMAS
T-CELL AND NATURAL KILLER-CELL LYMPHOMAS
HODGKIN LYMPHOMAS
SELECTED REFERENCES

CHAPTER 87: MOLECULAR GENETIC ASPECTS OF NON-HODGKIN LYMPHOMAS

INTRODUCTION
OVERVIEW OF METHODOLOGIES
RECURRENT MOLECULAR ABERRANCIES IN B-CELL AND T-CELL NEOPLASMS
SELECTED REFERENCES

CHAPTER 88: NON-HODGKIN LYMPHOMA IN ADULTS

HISTORICAL PERSPECTIVE
EPIDEMIOLOGY
PRELYMPHOMATOUS CONDITIONS
CLINICAL FEATURES AT PRESENTATION
STAGING
PROGNOSTIC FACTORS
THERAPEUTIC PRINCIPLES
INDOLENT LYMPHOMAS
MANTLE CELL LYMPHOMA
AGGRESSIVE LYMPHOMAS
THERAPY FOR LOCALIZED LARGE CELL LYMPHOMA
CHEMOTHERAPY
SALVAGE CHEMOTHERAPY
NOVEL AGENTS
HIGHLY AGGRESSIVE LYMPHOMAS
MANAGEMENT OF EXTRANODAL LYMPHOMAS
ROLE OF HEMATOPOIETIC CELL TRANSPLANTATION
SPECIAL AREAS
FUTURE DEVELOPMENTS
SELECTED REFERENCES

CHAPTER 89: NON-HODGKIN LYMPHOMA IN CHILDREN

INTRODUCTION
EPIDEMIOLOGY
PATHOLOGIC SPECIMEN EVALUATION
CLASSIFICATION
CLINICAL FEATURES
MANAGEMENT
LONG-TERM SEQUELAE
FUTURE DIRECTIONS
SELECTED REFERENCES

CHAPTER 90: CHRONIC LYMPHOCYTIC LEUKEMIA

PATHOPHYSIOLOGY
CLINICAL FINDINGS
DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
INVESTIGATIONS AND STAGING
PROGNOSIS
TREATMENT
SYSTEMIC COMPLICATIONS REQUIRING THERAPY
TRANSFORMATION OF CHRONIC LYMPHOCYTIC LEUKEMIA
SECOND MALIGNANCIES
SUMMARY OF THERAPY
SELECTED REFERENCES

CHAPTER 91: HAIRY CELL LEUKEMIA

INCIDENCE AND ETIOLOGY
PATHOGENESIS
CLINICAL FINDINGS
LABORATORY FINDINGS
DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
TREATMENT
SUMMARY
SECOND MALIGNANCIES
PROGNOSIS
SELECTED REFERENCES

CHAPTER 92: CUTANEOUS T-CELL LYMPHOMA: MYCOSIS FUNGOIDES AND SÉZARY SYNDROME

HISTORICAL PERSPECTIVE AND PATHOPHYSIOLOGY
EPIDEMIOLOGY
CLINICAL PRESENTATION
CLASSIFICATION OF CUTANEOUS T-CELL LYMPHOMAS
DIAGNOSTIC EVALUATION
DIFFERENTIAL DIAGNOSIS
STAGING
PROGNOSIS
THERAPY
SUMMARY AND FUTURE INVESTIGATIONS
WEB SITES
SELECTED REFERENCES

CHAPTER 93: HODGKIN LYMPHOMA IN ADULTS

EARLY HISTORY
EPIDEMIOLOGY AND ETIOLOGY
HISTOPATHOLOGY
LYMPHOCYTE-PREDOMINANT HODGKIN LYMPHOMA
CLINICAL EVALUATION
OVERVIEW AND HISTORICAL PERSPECTIVE OF RADIATION THERAPY
OVERVIEW AND HISTORICAL PERSPECTIVE OF CHEMOTHERAPY
FRONT-LINE THERAPY FOR ADVANCED-STAGE HODGKIN LYMPHOMA
THERAPY FOR RELAPSED OR REFRACTORY HODGKIN LYMPHOMA
HODGKIN LYMPHOMA IN THE ELDERLY
HODGKIN LYMPHOMA AND HUMAN IMMUNODEFICIENCY VIRUS
HODGKIN LYMPHOMA AND PREGNANCY
THERAPY FOR LYMPHOCYTE-PREDOMINANT HODGKIN LYMPHOMA
FOLLOW-UP OF PATIENTS WITH HODGKIN LYMPHOMA
SUMMARY
SELECTED REFERENCES

CHAPTER 94: HODGKIN LYMPHOMA IN CHILDREN

INTRODUCTION
EPIDEMIOLOGY
CLINICAL CONSIDERATIONS
INITIAL TREATMENT FOR PEDIATRIC HODGKIN LYMPHOMA
TREATMENT FOR RELAPSED HODGKIN LYMPHOMA
ADVERSE LONG-TERM OUTCOMES OF THERAPY
CONCLUSIONS
SELECTED REFERENCES

SECTION 5: PLASMA CELL DYSCRASIAS

CHAPTER 95: PRACTICAL APPROACH TO EVALUATION OF MONOCLONAL GAMMOPATHIES

CLASSIFICATION OF MONOCLONAL IMMUNOGLOBULIN DISORDERS
PROTEIN ELECTROPHORESIS
IDENTIFICATION OF THE TYPE AND QUANTITATION OF THE MONOCLONAL PROTEIN
QUANTITATION OF IMUNOGLOBULINS
FREE LIGHT CHAIN ASSAY
URINE EVALUATION
CONGO RED STAIN

CHAPTER 96: MOLECULAR GENETIC ASPECTS OF PLASMA CELL DISORDERS

INTRODUCTION
MULTIPLE MYELOMA IS A PLASMA CELL TUMOR OF POSTGERMINAL CENTER B CELLS
MULTIPLE MYELOMA IS ALMOST ALWAYS PRECEDED BY AN ASYMPTOMATIC MONOCLONAL GAMMOPATHY
A SELECTION FOR GENETIC MUTATIONS THAT ALLOW INDEPENDENCE FROM THE BONE MARROW MICROENVIRONMENT
CONCLUSION
SELECTED REFERENCES

CHAPTER 97: MONOCLONAL GAMMOPATHIES OF UNDETERMINED SIGNIFICANCE AND SMOLDERING MULTIPLE MYELOMA

INTRODUCTION
MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE
SMOLDERING MULTIPLE MYELOMA
VARIANTS OF MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE
ASSOCIATION OF MONOCLONAL GAMMOPATHY WITH OTHER DISEASES
SELECTED REFERENCES

CHAPTER 98: MULTIPLE MYELOMA

HISTORY
INCIDENCE AND EPIDEMIOLOGY
CLINICAL MANIFESTATIONS
DIAGNOSIS
SYSTEMIC THERAPY FOR MULTIPLE MYELOMA
RADIATION THERAPY
PROGNOSIS AND PATHOGENESIS
SPECIAL CASES OF MYELOMA
TREATMENT OF COMPLICATIONS AND SUPPORTIVE CARE
DIFFERENTIAL DIAGNOSIS
SELECTED REFERENCES

CHAPTER 99: IMMUNOGLOBULIN LIGHT-CHAIN AMYLOIDOSIS (PRIMARY AMYLOIDOSIS)

HISTORY
CLASSIFICATION
SYMPTOMS AND SIGNS
DIAGNOSIS OF AMYLOIDOSIS
DIFFERENTIATING AMYLOIDOSIS FROM OTHER FORMS OF AMYLOIDOSIS
PRESENTATION AND CLINICAL FEATURES
PROGNOSTIC FEATURES
TREATMENT
CONCLUSION
SELECTED REFERENCES

CHAPTER 100: WALDENSTRÖM MACROGLOBULINEMIA

INTRODUCTION
EPIDEMIOLOGY
DIAGNOSIS AND LABORATORY TESTING
IgM MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE AND SMOLDERING WALDENSTRÖM MACROGLOBULINEMIA
PATHOLOGY
BIOLOGY AND GENETICS
CLINICAL FEATURES
PROGNOSIS
CONVENTIONAL TREATMENTS AND MANAGEMENT
HIGH-DOSE CHEMOTHERAPY WITH STEM CELL SUPPORT
OTHER THERAPEUTIC INTERVENTIONS
TOXICITY OF STANDARD THERAPY
MAYO CLINIC TREATMENT RECOMMENDATIONS
CONCLUSIONS
SELECTED REFERENCES

CHAPTER 101: POEMS SYNDROME, CRYOGLOBULINEMIA, AND HEAVY-CHAIN DISEASE

INTRODUCTION
POEMS SYNDROME
ETIOLOGY OF POEMS SYNDROME
DIAGNOSIS OF POEMS SYNDROME
CLINICAL PRESENTATION OF POEMS SYNDROME
TREATMENT OF POEMS SYNDROME
CRYOGLOBULINEMIA
EPIDEMIOLOGY OF CRYOGLOBULINEMIA
ETIOLOGY OF CRYOGLOBULINEMIA
CLINICAL PRESENTATION OF CRYOGLOBULINEMIA
DIAGNOSIS OF CRYOGLOBULINEMIA
TREATMENT OF CRYOGLOBULINEMIA
HEAVY-CHAIN DISEASE
SELECTED REFERENCES

PART VIII: Transplantation

CHAPTER 102: HEMATOPOIETIC CELL TRANSPLANTATION

HISTORICAL PERSPECTIVE
HEMATOPOIETIC STEM CELLS
SELECTION OF STEM CELL SOURCE
COLLECTION OF HEMATOPOIETIC STEM CELLS FOR TRANSPLANTATION
ABO INCOMPATIBILITY
CONDITIONING REGIMENS
ENGRAFTMENT
REGIMEN-RELATED TOXICITIES
GRAFT-VERSUS-HOST DISEASE
GRAFT-VERSUS-TUMOR EFFECT
IMMUNE RECONSTITUTION
SELECTED REFERENCES

CHAPTER 103: HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR NONMALIGNANT DISORDERS

INTRODUCTION
CONDITIONS SUITABLE FOR HEMATOPOIETIC STEM CELL TRANSPLANTATION
HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR PRIMARY IMMUNODEFICIENCY DISORDERS
HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR FANCONI ANEMIA
LATE EFFECTS
LONG-TERM TOXICITIES OF CONDITIONING
CONCLUSION
SELECTED REFERENCES

CHAPTER 104: ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION (HCT) FOR HEMATOLOGIC MALIGNANCIES

INTRODUCTION
CURRENT OUTCOME DATA
SELECTED REFERENCES

CHAPTER 105: GRAFT-VERSUS-HOST DISEASE AND GRAFT-VERSUS-TUMOR RESPONSE

INTRODUCTION
ACUTE GRAFT-VERSUS-HOST DISEASE
EPIDEMIOLOGY
RISK FACTORS
PROGNOSIS
CLINICAL MANIFESTATIONS OF ACUTE GRAFT-VERSUS-HOST DISEASE
PREVENTION
PRIMARY AND SECONDARY THERAPY OF ACUTE GRAFT-VERSUS-HOST DISEASE
SUPPORTIVE CARE
CONCLUSION
CHRONIC GRAFT-VERSUS-HOST DISEASE
CLASSIFICATION AND SEVERITY GRADING
PROGNOSTIC VARIABLES
PREVENTION OF CHRONIC GRAFT-VERSUS-HOST DISEASE
CHRONIC GRAFT-VERSUS-HOST DISEASE TREATMENT
ASSESSMENT OF THERAPEUTIC RESPONSE
SUPPORTIVE CARE
CONCLUSION
GRAFT-VERSUS-TUMOR RESPONSE
SELECTED REFERENCES

CHAPTER 106: LATE EFFECTS AFTER TRANSPLANTATION

INTRODUCTION
CARDIOVASCULAR EVENTS AND METABOLIC SYNDROME
SECONDARY MALIGNANCIES
LATE PULMONARY COMPLICATIONS
INFECTION AND IMUNE RECONSTITUTION
BONE MINERAL DENSITY LOSS AND AVASCULAR OSTEONECROSIS
OCULAR COMPLICATIONS IN LONG-TERM SURVIVORS AFTER HEMATOPOIETIC CELL TRANSPLANTATION
ENDOCRINE AND SEXUAL DYSFUNCTIONS IN LONG-TERM SURVIVORS
CHRONIC KIDNEY DISEASES: MONITORING AND MANAGEMENT GUIDELINES
GASTROINTESTINAL AND LIVER PROBLEMS
IRON OVERLOAD IN LONG-TERM SURVIVORS
FEMALE LONG-TERM SURVIVORS AND RELATED ISSUES
QUALITY OF LIFE AND SOCIAL CHANGES IN LONG-TERM SURVIVORS
CONCLUSION
SELECTED REFERENCES

INDEX

Related Book: Wintrobe’s Clinical Hematology 14th Edition PDF

Editorial Reviews

Review

BMA Medical Book Awards 2014 
FIRST PRIZE- Wintrobe’s Clinical Hematology, 13th Edition

“It is a pleasure to have this book on my desktop- I am unlikely to part with it long enough to put it on a shelf. This book has been a favourite of mine for many years and yet this update has moved it on to another plane. The presentation is excellent and the book is one to be coveted. The online access and the regular planned updates will be a valuable resource to keep this book at the forefront of its field. There is a need to have a unifying text and there are relatively few books that manage to achieve this. I, therefore, feel that this fills a gap in the market. The book is very easy to read and therefore ideal for individuals “dipping in” to read sections or to look up specific sections. The illustrations make it particularly appealing. The style lends itself well to trainees in the specialties it is designed for, and the referencing and online resource make it useful for specialists that need to span a broad cross-section of haematology. This is a great book and its new design makes it particularly appealing. I particularly like having everything in a single volume and the indexing is very clear.”

– BMA Medical Book Awards 2014 Reviewer

4 START REVIEW FROM DOODY’S REVIEW SERVICE!

“The 13th edition is a justified replacement of the previous edition due to the advances in the understanding of the biology, diagnostic tests, and treatment options in the intervening years.”

– Robert Chen, MD, City of Hope on belaf of Doody’s Review Serivce

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