Lanzkowsky's Manual of Pediatric Hematology and Oncology

 
 
Academic Press
  • 6. Auflage
  • |
  • erschienen am 22. April 2016
  • |
  • 788 Seiten
 
E-Book | ePUB mit Adobe DRM | Systemvoraussetzungen
E-Book | PDF mit Adobe DRM | Systemvoraussetzungen
978-0-12-801674-9 (ISBN)
 

Lanzkowsky's Manual of Pediatric Hematology and Oncology, Sixth Edition, is a comprehensive book on patient management, replete with algorithms and flow diagrams on diagnosis and management. Reflecting the considerable advances in the treatment and management of hematologic and oncologic diseases in children, the sixth edition of this successful clinical manual has been entirely updated to incorporate all current treatment protocols, new drugs, and management approaches. Its concise and easy-to-read format will enable readers to make accurate diagnoses and permit them to treat patients without having to reference larger medical textbooks.

Based on the new standards of genetic classification and prognostic information that have arisen in the past five years, the sixth edition includes two new chapters (Diagnostic, Molecular, and Genomic Methodologies for the Hematologist, Transfusion Medicine) and several new expanded chapters that were previously sections in consolidated chapters (Myelodysplasia, Myeloid Leukemias, Lymphoid Leukemias, Hemolytic Anemia, and Disorders of Coagulation).


  • Presents a concise, systematic approach to all pediatric hematologic and oncologic disorders in one manual
  • Offers an alternative to bigger references which only cover either oncologic or hematologic disorders in twice as many pages
  • Presents an easy-to-read format: multiple tables, charts, and flow-diagrams for diagnosis and management of pediatric hematologic and oncologic disorders
  • Includes 2 new chapters and several expanded chapters: Diagnostic, Molecular and Genomic Methodologies for the Hematologist, Transfusion Medicine, Myelodysplasia, Myeloid Leukemias, and Lymphoid Leukemias
  • Englisch
  • San Diego
  • |
  • USA
Elsevier Science
  • 11,74 MB
978-0-12-801674-9 (9780128016749)
0128016744 (0128016744)
weitere Ausgaben werden ermittelt
  • Front Cover
  • Lanzkowsky's Manual of Pediatric Hematology and Oncology
  • Copyright Page
  • Dedication
  • Contents
  • List of Contributors
  • About the Editors
  • Philip Lanzkowsky
  • Jeffrey M. Lipton
  • Jonathan D. Fish
  • Preface to the Sixth Edition
  • Preface to the Fifth Edition
  • Preface to the Fourth Edition
  • Preface to the Third Edition
  • Preface to the Second Edition
  • Preface to the First Edition
  • Introduction: Historic Perspective
  • Reflection on 60 Years of Progress in Pediatric Hematology-Oncology
  • 1 Diagnostic Molecular and Genomic Methodologies for the Hematologist/Oncologist
  • Clinical Molecular and Genomic Methodologies
  • Linkage Analysis
  • Fluorescent In Situ Hybridization
  • Array CGH
  • Genome-Wide or Focused SNP Arrays
  • Multiplex Ligation-Dependent Probe Amplification
  • Targeted PCR Analysis
  • Focused Sanger and Gene Panel Sequencing
  • Whole-Genome or -Exome Sequencing
  • Interpreting and Evaluating the Results from Clinical Genetic Testing
  • Further Reading and References
  • 2 Hematologic Manifestations of Systemic Illness
  • Hematologic Manifestations of Diseases Related to Various Organs
  • Heart
  • Cardiac Anomalies and Hyposplenism
  • Infective Endocarditis
  • Coagulation Abnormalities
  • Platelet Abnormalities
  • Polycythemia
  • Gastrointestinal Tract
  • Esophagus
  • Stomach
  • Small Bowel
  • Lower Gastrointestinal Tract
  • Pancreas
  • Liver
  • Anemia
  • Coagulation Abnormalities
  • Factor I (Fibrinogen)
  • Factors II, VII, IX, and X (Vitamin K-Dependent Factors)
  • Factor V
  • Factor VIII
  • Protein C, Protein S, and Antithrombin III
  • Tissue plasminogen activator (TPA) and alpha-2-antiplasmin
  • a2-Macroglobulin and plasmin activator inhibitor
  • Kidneys
  • Treatment
  • Endocrine Glands
  • Thyroid
  • Adrenal Glands
  • Lungs
  • Skin
  • Mast Cell Disease
  • Eczema and Psoriasis
  • Dermatitis Herpetiformis
  • Dyskeratosis Congenita
  • Hereditary Hemorrhagic Telangiectasia
  • Ehlers-Danlos Syndrome
  • Chronic Illness
  • Inflammatory Bowel Disease as a Model for Anemia of Chronic Illness
  • Connective Tissue Diseases
  • Rheumatoid Arthritis
  • Felty Syndrome
  • Systemic Lupus Erythematous
  • Polyarteritis Nodosa
  • Wegener Granulomatosis
  • Kawasaki Syndrome
  • Henoch-Schönlein Purpura
  • Infections
  • Anemia
  • White Cell Alterations
  • Clotting Abnormalities
  • Thrombocytopenia
  • Viral and Bacterial Illnesses Associated with Marked Hematologic Sequelae
  • Parvovirus
  • Epstein-Barr Virus
  • Human Immunodeficiency Virus
  • Thrombocytopenia
  • Anemia and Neutropenia
  • Coagulation Abnormalities
  • Role of Hematopoietic Growth Factors in Acquired Immunodeficiency Syndrome
  • Cancers in Children with Human Immunodeficiency Virus Infection
  • Treatment of HIV Infection-Related Lymphomas
  • Proliferative Lesions of Mucosa-Associated Lymphoid Tissue
  • Treatment of Low-Grade MALT Lymphoma
  • Leiomyosarcomas and Leiomyomas
  • Kaposi Sarcoma
  • Leukemias
  • Miscellaneous Tumors
  • Infections
  • Torchs
  • Salmonella typhi
  • Acute Infectious Lymphocytosis
  • Bartonellosis
  • Tuberculosis
  • Leptospirosis (Weil Disease)
  • Parasitic Illnesses Associated with Marked Hematologic Sequelae
  • Malaria
  • Babesiosis
  • Leishmaniasis
  • Hookworm
  • Tapeworm
  • Trypanosomiasis
  • Lead Intoxication
  • Nutritional Disorders
  • Protein-Calorie Malnutrition
  • Scurvy
  • Anorexia Nervosa
  • Bone Marrow Infiltration
  • Gaucher Disease
  • Pathogenesis
  • Diagnosis
  • Further Evaluation
  • Treatment
  • Response to Therapy
  • Niemann-Pick Disease
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • "Foam Cells" in Bone Marrow
  • Cystinosis
  • Diagnosis
  • Infantile Malignant Osteopetrosis (Marble Bone Disease)
  • Severe Form (Autosomal Recessive)
  • Treatment
  • Mild Form (Autosomal Dominant)
  • Neoplastic Disease
  • Further Reading and References
  • 3 Classification and Diagnosis of Anemia in Children
  • Classification and Diagnosis
  • Further Reading and References
  • 4 Lymphadenopathy and Diseases of the Spleen
  • Lymphadenopathy
  • History
  • Age
  • Location
  • Localized or Generalized
  • Size
  • Character
  • Diagnosis of Lymphadenopathy
  • Diseases of the Spleen
  • Asplenia
  • Congenital Polysplenia
  • Accessory Spleen
  • Splenosis
  • Sequestration of Spleen
  • Splenoptosis (Splenic Visceroptosis)
  • Splenomegaly
  • Diagnostic Approach to Splenomegaly
  • Detailed History
  • Physical Examination
  • Laboratory Investigations
  • Surgery Involving Spleen
  • Further Reading and References
  • 5 Anemia During the Neonatal Period
  • Hemorrhage
  • Prenatal Blood Loss
  • Transplacental Fetomaternal
  • Intraplacental and Retroplacental
  • Twin-to-Twin Transfusion
  • Intranatal Blood Loss
  • Postnatal Blood Loss
  • Clinical and Laboratory Findings of Anemia due to Hemorrhage
  • Treatment
  • Hemolytic Anemia
  • Congenital Erythrocyte Defects
  • Infantile Pyknocytosis
  • Anemia due to Hemoglobinopathies
  • Gamma Chain Defects
  • Beta Chain Defects
  • Alpha Chain Defects
  • Acquired Erythrocyte Defects
  • Immune Hemolytic Anemia
  • Rh Isoimmunization
  • Clinical Features
  • Laboratory Findings
  • Management
  • Antenatal
  • Postnatal
  • Prevention of Rh Hemolytic Disease
  • ABO Isoimmunization
  • Clinical Features
  • Diagnosis
  • Treatment
  • Late-Onset Anemia in Immune Hemolytic Anemia
  • Nonimmune Hemolytic Anemia
  • Vitamin E Deficiency
  • Clinical Findings
  • Diagnosis
  • Failure of Red Cell Production
  • Congenital
  • Acquired
  • Viral Diseases
  • Anemia of Prematurity
  • Clinical Features
  • Treatment
  • Physiologic Anemia
  • Diagnostic Approach to Anemia in the Newborn
  • Further Reading and References
  • 6 Iron-Deficiency Anemia
  • Prevalence
  • Etiologic Factors
  • Diet
  • Food Iron Content
  • Growth
  • Blood Loss
  • Impaired Absorption
  • Clinical Features
  • Iron-Refractory Iron-Deficiency Anemia
  • Non-hematological Manifestations
  • Diagnosis
  • Stages of Iron Depletion
  • Differential Diagnosis
  • Treatment
  • Nutritional Counseling
  • Oral Iron Medication
  • Parenteral Therapy
  • Indications
  • Intramuscular
  • Dose
  • Intravenous
  • Side Effects
  • Contraindications to Parenteral Iron Therapy
  • Blood Transfusion
  • Partial Exchange Transfusion
  • Further Reading and References
  • 7 Megaloblastic Anemia
  • Vitamin B12 (Cobalamin) Deficiency
  • Absorption and Metabolism
  • Nutritional Deficiency
  • Defective Absorption
  • Gastric Acidity and Peptic Activity Deficiency
  • Intrinsic Factor Deficiency
  • Defective Cobalamin Transport by Ileal Enterocyte Receptors for the Intrinsic Factor-Cobalamin Complex (Imerslund-Gräsbeck ...
  • Defective Transport
  • Transcobalamin II Deficiency (OMIM 275350)
  • Diagnosis
  • Treatment
  • Partial Deficiency of Transcobalamin I (Haptocorrin Deficiency) (OMIM 193090)
  • Disorders of Metabolism: Congenital
  • Adenosylcobalamin Deficiency CblA (OMIM 251100) and CblB (OMIM 251110) Diseases
  • Methylmalonyl-CoA Mutase (mut, mut2) Deficiency
  • Methylcobalamin Synthesis Deficiency: CblE (OMIM 236270) and CblG (OMIM 250940) Diseases
  • Combined Adenosylcobalamin and Methylcobalamin Deficiencies CblC (OMIM 277400), CblD (OMIM 277410), and CblF (OMIM 277380) ...
  • Disorders of Metabolism: Acquired
  • Folic Acid Deficiency
  • Absorption and Metabolism
  • Acquired Folate Deficiency
  • Inborn Errors of Folate Transport and Metabolism
  • Hereditary Folate Malabsorption (OMIM 229050)
  • Clinical Presentation
  • Treatment
  • Methylenetetrahydrofolate Reductase Deficiency (OMIM 236250)
  • Clinical Findings
  • Diagnosis
  • Prognosis
  • Treatment
  • Glutamate Formiminotransferase Deficiency (OMIM 229100)
  • Functional Methionine Synthase Deficiency (OMIM 250940)
  • Other Megaloblastic Anemias
  • Clinical Features of Cobalamin and Folate Deficiency
  • Diagnosis of Cobalamin and Folate Deficiency
  • Treatment
  • Vitamin B12 Deficiency
  • Folic Acid Deficiency
  • Response to Folic Acid Treatment
  • Further Reading and References
  • 8 Bone Marrow Failure
  • Aplastic Anemia
  • Acquired Aplastic Anemia
  • Definition
  • Pathophysiology
  • Clinical Manifestations
  • Laboratory Investigations
  • Treatment
  • Supportive Care
  • Specific Therapy
  • Hematopoietic Stem Cell Transplantation
  • Immunosuppressive Therapy
  • Antithymocyte Globulin
  • Cyclosporine (CSA) Preparations
  • Hematopoietic Growth Factors
  • Treatment Choices and Long-Term Follow-Up
  • Salvage Therapy
  • Long-Term Sequelae and Outcomes for SAA
  • Treatment of Moderate Aplastic Anemia
  • Inherited Bone Marrow Failure Syndromes
  • Fanconi Anemia
  • Pathophysiology
  • Clinical Manifestations
  • Diagnosis
  • Complications
  • Differential Diagnosis
  • Management
  • Treatment
  • Prognosis
  • Dyskeratosis Congenita
  • Pathophysiology
  • Genetics
  • Clinical Manifestations
  • Treatment
  • Congenital Aplastic Anemias of Unknown Inheritance
  • Diamond-Blackfan Anemia (Congenital Pure Red Cell Aplasia)
  • Pathophysiology
  • Genetics
  • Clinical Manifestations
  • Diagnosis
  • Differential Diagnosis
  • Treatment
  • Prognosis
  • Transient Erythroblastopenia of Childhood
  • Congenital Dyserythropoietic Anemia
  • Clinical Manifestations
  • Differential Diagnosis
  • Treatment
  • Sideroblastic Anemias (Mitochondrial Diseases with Bone Marrow Failure Syndromes)
  • Laboratory Findings
  • Pathophysiology
  • Treatment
  • Severe Congenital Neutropenia and Kostmann Syndrome
  • Epidemiology
  • Incidence
  • Pathogenesis and Genetics
  • Clinical Manifestations and Laboratory Investigations
  • Treatment
  • Prognosis
  • Reticular Dysgenesis
  • Further Reading and References
  • 9 General Considerations of Hemolytic Diseases, Red Cell Membrane, and Enzyme Defects
  • Clinical Features of Hemolytic Disease
  • Laboratory Findings
  • Accelerated Hemoglobin Catabolism
  • Markers of Extravascular Hemolysis
  • Markers of Intravascular Hemolysis
  • Increased Erythropoiesis
  • Membrane Defects
  • Structure of the Red Cell Membrane
  • Red Cell Membrane Disorders
  • Hereditary Spherocytosis
  • Genetics
  • Pathogenesis
  • Hematologic Features
  • Biochemical Features
  • Clinical Features
  • Classification
  • Diagnosis
  • Complications
  • Treatment
  • Hereditary Elliptocytosis
  • Genetics
  • Pathogenesis
  • Hematologic Features
  • Clinical Features
  • Classification
  • Treatment
  • Hereditary Pyropoikilocytosis
  • Genetics
  • Pathogenesis
  • Biochemical and Biophysical Features
  • Clinical Features
  • Differential Diagnosis
  • Treatment
  • Hereditary Stomatocytosis
  • Definition and Genetics
  • Etiology
  • Clinical Features
  • Overhydrated Stomatocytosis
  • Dehydrated Stomatocytosis
  • Differential Diagnosis
  • Treatment
  • Hereditary Acanthocytosis
  • Definition
  • Genetics
  • Clinical Features
  • Diagnosis
  • Differential Diagnosis
  • Paroxysmal Nocturnal Hemoglobinuria
  • Pathogenesis
  • Mechanism of Hemolysis and Hemoglobinuria in PNH
  • Mechanism of Hypercoagulable State
  • Mechanism of Defective Hematopoiesis
  • Clinical Manifestations
  • Course of the Disease
  • Complications
  • Intravascular Hemolysis (DAT Negative)
  • Venous Thrombosis
  • Defective Hematopoiesis
  • Infectious
  • Other
  • Diagnosis
  • Management
  • Hematopoietic Stem Cell Transplantation
  • Ecluzumab
  • Immunosuppressive Therapy
  • Use of Hematopoietic Growth Factor
  • Supportive Therapy
  • Enzyme Defects
  • PK Deficiency
  • Genetics
  • Pathogenesis
  • Hematology
  • Clinical Features
  • Treatment
  • Other Enzyme Deficiencies
  • Glucose-6-Phosphate Dehydrogenase Deficiency
  • Genetics
  • Pathogenesis
  • Clinical Features
  • Treatment
  • Other Defects of Glutathione Metabolism
  • Glutathione Reductase
  • Glutamyl Cysteine Synthetase
  • Glutathione Synthetase
  • Glutathione Peroxidase
  • Further Reading and References
  • 10 Extracorpuscular Hemolytic Anemia
  • Immune Hemolytic Anemia
  • Warm AIHA
  • Clinical Features
  • Laboratory Findings
  • Management
  • Treatment
  • Blood Transfusion
  • Corticosteroid Therapy
  • Intravenous Gammaglobulin
  • Rituximab
  • Plasmapheresis
  • Immunomodulating Agents
  • Antimetabolites
  • Alkylating Agents
  • Mitotic Inhibitors
  • Splenectomy
  • Giant Cell Hepatitis and DAT-Positive AIHA
  • Clinical Findings
  • Prognosis
  • Laboratory Findings
  • Treatment
  • Cold AIHA
  • Clinical Features
  • Treatment
  • Paroxysmal Cold Hemoglobinuria Due to Donath-Landsteiner Cold Hemolysin
  • Clinical Features
  • Laboratory Findings
  • Treatment
  • NonImmune Hemolytic Anemia
  • Microangiopathic Hemolytic Anemia
  • Diagnosis
  • Hypersplenism
  • Wilson Disease
  • Further Reading and References
  • 11 Hemoglobinopathies
  • Sickle Cell Disease
  • Incidence
  • Genetics
  • Pathophysiology
  • Clinical Features
  • Hematology
  • Acute Complications
  • Chronic Complications and End-Organ Damage
  • Diagnosis
  • Prognosis
  • Management
  • Sickle Cell Trait (Heterozygous Form, AS)
  • Hematology
  • Clinical Features
  • Significance
  • Hemoglobin C
  • Basic Features and Pathology
  • HbC Disease (Homozygous CC)
  • Hematology
  • Clinical Features
  • HbC Trait (Heterozygous Form, AC)
  • Hemoglobin SCD
  • Hematology
  • Clinical Features
  • HbS/ß-Thalassemia
  • Hemoglobin E
  • Unstable Hemoglobins
  • Thalassemias
  • Basic Features
  • ß-Thalassemia
  • a-Thalassemia
  • ß-Thalassemia: Homozygous or Doubly Heterozygous Forms (Major and Intermedia)
  • Pathogenesis
  • Sequelae
  • Hematology
  • Biochemistry
  • Clinical Features
  • Complications
  • Causes of Death
  • Management
  • Transfusion Therapy
  • Monitoring Iron Overload
  • Chelation Therapy
  • Splenectomy
  • Supportive Care
  • Pharmacologic Enhancement of HbF Synthesis
  • Hematopoietic Stem Cell transplantation
  • Gene Therapy
  • Management of the Acutely Ill Thalassemic Patient
  • ß-Thalassemia Intermedia
  • Clinical Features
  • Management
  • ß-Thalassemia Minor or Trait (Heterozygous ß0 or ß+)
  • Clinical Features
  • a-Thalassemias
  • Further Reading and References
  • 12 Polycythemia
  • Polycythemia (Erythrocytosis) in the Newborn
  • Symptoms
  • Laboratory Findings
  • Treatment
  • Polycythemia in Childhood
  • Polycythemia Vera
  • Pathophysiology
  • Clinical Features
  • Diagnosis
  • Treatment
  • Primary Familial and Congenital Polycythemia
  • Congenital Polycythemia Due to Altered Hypoxia Sensing with Normal P50
  • Chuvash Polycythemia and Other von Hippel Lindau Mutations
  • Clinical Manifestations
  • HIF2a Mutations
  • Prolyl Hydroxylase Domain-2 (PHD2) Mutations
  • Congenital Polycythemia Due to Altered Hypoxia Sensing with Decreased P50
  • High-Affinity Hemoglobinopathies
  • 2,3-Bisphosphoglycerate Deficiency
  • Methemoglobinemia
  • Other Causes of Polycythemia
  • Diagnostic Approach
  • Further Reading and References
  • 13 Disorders of White Blood Cells
  • Quantitative Disorders of Leukocytes
  • Leukocytosis
  • Leukopenias
  • Neutropenia
  • Clinical Manifestations
  • Decreased Production or Intrinsic Defects of Neutrophils
  • Benign Ethnic Neutropenia
  • Neutropenia Associated with X-linked Agammaglobulinemia
  • Clinical Manifestations
  • Treatment
  • Neutropenia Associated with Autosomal Recessive Agammaglobulinemia
  • Treatment
  • Neutropenia Associated with Abnormal Cellular Immunity in Cartilage Hair Hypoplasia
  • Clinical Manifestations
  • Treatment
  • Neutropenia Associated with Common Variable Immunodeficiency
  • Clinical Manifestations
  • Treatment
  • Myelokathexis and WHIM Syndromes
  • Genetics
  • Clinical Manifestations
  • Treatment
  • Selective IgA Deficiency and Neutropenia
  • Dubowitz Syndrome
  • Neutropenia Associated with Hyperimmunoglobulin M Syndrome
  • Genetics
  • Clinical Manifestations
  • Treatment
  • Neutropenia Associated with Metabolic Diseases
  • Glycogen Storage Disease Type 1b
  • Clinical Manifestations
  • Treatment
  • Barth Syndrome
  • Treatment
  • Bone Marrow Disease
  • Increased Destruction or Extrinsic Defects of Neutrophils
  • Drug-Induced Neutropenia
  • Treatment
  • Immune Neutropenia
  • Neonatal Immune Neutropenia-Alloimmune and Autoimmune
  • Clinical Manifestations
  • Treatment
  • Autoimmune Neutropenia
  • Primary Autoimmune Neutropenia
  • Diagnosis
  • Prognosis
  • Treatment
  • Secondary Autoimmune Neutropenia
  • Treatment
  • Autoimmune Lymphoproliferative Syndrome
  • NonImmune Neutropenia
  • Chronic Idiopathic Neutropenia
  • Clinical Manifestations
  • Treatment
  • Infections
  • Hypersplenism
  • Investigations in Neutropenia
  • Management of Neutropenia
  • Eosinophils
  • Eosinophilia
  • Classification of Eosinophilia
  • (Idiopathic) Hypereosinophilic Syndrome
  • Definition
  • Clinical Presentation
  • Clinical Manifestations
  • Treatment
  • Nonidiopathic Eosinophilia
  • Primary Clonal Eosinophilic Disorders
  • PDGFRA, PDGFRB, FGFR1 Rearranged Neoplasms
  • Secondary Clonal Eosinophilic Disorders
  • Secondary Eosinophilic Clonal Disorders
  • Eosinophilia in Newborn Period
  • Familial Eosinophilia
  • Lymphocytes
  • Atypical Lymphocytosis
  • Infectious Mononucleosis
  • Differential Diagnosis
  • Lymphopenia
  • Disorders of Leukocyte Function
  • Disorders of Adhesion and Chemotaxis
  • Leukocyte Adhesion Disorders
  • Leukocyte Adhesion Deficiency Type I
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Leukocyte Adhesion Deficiency Type II
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Leukocyte Adhesion Deficiency Type III
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Rac2 GTPase Mutation
  • Treatment
  • Hyperimmunoglobulin E Syndrome (Job Syndrome)
  • Genetics
  • Clinical Manifestations
  • Laboratory Findings
  • Treatment
  • Localized Aggressive Periodontitis in Children (Formerly Known as Localized Juvenile Periodontitis)
  • Clinical Manifestations
  • Genetics
  • Laboratory Findings
  • Treatment
  • Disorders of Neutrophil Granules
  • Chédiak-Higashi Syndrome
  • Genetics
  • Clinical Manifestations
  • Hematologic Features
  • Diagnosis
  • Treatment
  • Neutrophil-Specific Granule Deficiency
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Disorders of Oxidative Metabolism
  • Chronic Granulomatous Disease
  • Pathogenesis
  • Genetics
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Prognosis
  • Myeloperoxidase Deficiency
  • Diagnosis
  • Treatment
  • Glutathione Pathway Disorders
  • Glutathione Synthetase Deficiency
  • Diagnosis
  • Treatment
  • Glutathione Reductase Deficiency
  • Glucose-6-Phosphate Dehydrogenase Deficiency in Leukocytes
  • Treatment
  • Neutrophil Production and Destruction in Newborn Infants
  • Neutrophil Production
  • Neutrophil Function in the Newborn
  • Chemotaxis
  • Vascular Rolling of Neutrophils
  • Vascular Endothelial Adhesion
  • Dynamics of Change of Shape
  • Phagocytosis
  • Respiratory Burst
  • Therapeutic Implications
  • Neonatal Preeclampsia-Associated Neutropenia
  • Treatment
  • Further Reading and References
  • 14 Disorders of Platelets
  • Thrombocytopenia in the Newborn
  • Neonatal Alloimmune Thrombocytopenia
  • Pathophysiology
  • Clinical Features
  • Diagnosis
  • Treatment
  • Management of Subsequent Pregnancies
  • Neonatal Autoimmune Thrombocytopenia
  • Pathophysiology
  • Diagnosis
  • Treatment
  • General Diagnostic Approach to a Newborn with Thrombocytopenia
  • Thrombocytopenia Associated with Hemolytic Disease of Fetus and Neonate
  • Thrombocytopenia Secondary to Chronic Fetal Hypoxia, Maternal Diabetes, Pregnancy-Induced Hypertension, or IUGR
  • Thrombocytopenia Secondary to Congenital Infections
  • Late-Onset Thrombocytopenia Secondary to Late-Onset Infections, Necrotizing Enterocolitis, or Thrombosis
  • Thrombocytopenia due to Aneuploidy
  • Rare Bone Marrow Disease or Inborn Errors of Metabolism
  • Metabolic Causes
  • Vascular Anomalies
  • Treatment
  • Inherited Thrombocytopenias
  • Bernard-Soulier Syndrome
  • Treatment
  • MYH9 Disorders
  • Wiskott-Aldrich Syndrome (WAS)
  • Pathophysiology
  • Clinical Manifestations
  • Hematologic Findings
  • Treatment
  • X-Linked Thrombocytopenia
  • Anemia and Thrombocytopenia with GATA1 Mutation
  • Thrombocytopenia with Absent Radii Syndrome
  • Treatment
  • Amegakaryocytic Thrombocytopenia (AMT)
  • Amegakaryocytic Thrombocytopenia with Radio-Ulnar Synostosis
  • Familial Platelet Syndrome with Predisposition to Acute Myelogenous Leukemia (FPS/AML)
  • Paris-Trousseau Syndrome
  • Congenital Hypoplastic Thrombocytopenia with Microcephaly
  • Thrombocytopenia Agenesis of Corpus Callosum Syndrome
  • Immune Thrombocytopenic Purpura (ITP)
  • Pathophysiology
  • Infections in ITP
  • Clinical Manifestations
  • Diagnosis
  • Treatment
  • Corticosteroids
  • Immune Globulin (IVIG)
  • Anti-D Therapy
  • Splenectomy
  • Rituximab
  • Thrombopoietic Agents
  • Plasmapheresis
  • Platelet Transfusions
  • Drugs that May Be Effective but Are Infrequently to Rarely Used in Pediatric ITP
  • Emergency Therapy
  • Chronic ITP
  • Other Causes of Thrombocytopenia
  • HIV-Associated Thrombocytopenia
  • Drug-Induced Thrombocytopenia
  • Heparin-Induced Thrombocytopenia
  • Thrombotic Microangiopathies
  • Thrombotic Thrombocytopenic Purpura
  • Acquired TTP
  • Congenital TTP (Upshaw-Shulman Syndrome)
  • Hemolytic-Uremic Syndrome
  • Atypical Hemolytic-Uremic Syndrome
  • Disseminated Intravascular Coagulation
  • Autoimmune Disorders
  • Cyanotic Congenital Heart Disease
  • Hypersplenism
  • Thrombocytosis
  • Primary Thrombocytosis
  • Essential Thrombocythemia
  • Hereditary Thrombocytosis
  • Treatment of Pediatric Primary Thrombocytosis
  • Antiplatelet Agents
  • Cytoreductive Therapy (Platelet-Lowering Drugs)
  • Qualitative Platelet Disorders
  • Defects in Platelet Receptor-Agonist interactions
  • Selective Impairments in Platelet Responsiveness to Epinephrine
  • Selective Impairment in Platelet Responsiveness to Collagen
  • Defects in Thromboxane A2
  • Selective Impairment in Platelet Response to ADP
  • Defects in Platelet Vessel-Wall Interaction
  • Bernard-Soulier Syndrome
  • Type 2B von Willebrand Disease and Platelet-Type (Pseudo-von Willebrand) Disease
  • Defects in Platelet-Platelet Interaction
  • Glanzmann Thrombasthenia
  • Disorders of Platelet Secretion
  • d-Storage Pool Deficiency
  • Hermansky-Pudlak Syndrome
  • Chédiak-Higashi Syndrome
  • a -Granule SPD (Gray Platelet Syndrome)
  • Quebec Platelet Disorder (Platelet Factor V Quebec)
  • Arthogryposis-Renal Dysfunction-Cholestasis
  • May-Hegglin Anomaly
  • Miscellaneous
  • Montreal Platelet Syndrome
  • Impaired Liberation of Arachidonic Acid Pathways
  • Cyclooxygenase and Thromboxane Synthetase Deficiency
  • Platelet Intracellular Signaling Defects
  • Kindlin-3 (Leukocyte Adhesion Defect III)
  • Dysregulated Calcium Signaling
  • Deficiency of Platelet Procoagulant Activity
  • Scott Syndrome
  • Isolated Defect in Membrane Vesiculation
  • Acquired Platelet Disorders
  • Medications
  • Renal Failure
  • Liver Disease
  • Management of Defects in Platelet Function
  • Inherited Vascular and Connective Tissue Disorders
  • Ehlers-Danlos Syndrome
  • Pseudoxanthoma Elasticum
  • Marfan's Syndrome
  • Osteogenesis Imperfecta
  • Hereditary Hemorrhagic Telangiectasia
  • Laboratory Evaluation of Platelets and Platelet Function
  • Examination of the Blood Smear
  • Bleeding Time
  • Closure Time
  • Platelet Aggregation in Platelet-Rich Plasma
  • Platelet Aggregation in Whole Blood
  • Nonthrombocytopenic Purpura
  • Anaphylactoid purpura
  • Infections
  • Drugs
  • Purpura Factitia
  • Gardner-Diamond Syndrome
  • Scurvy
  • Further Reading and References
  • 15 Disorders of Coagulation
  • Hemostatic Disorders
  • Physiology of Hemostasis
  • Relevant Components of Hemostasis
  • Primary Hemostatic Mechanism (Platelet Phase)
  • Fibrin Thrombus Formation
  • Platelet Vessel Interaction
  • Fibrinolysis
  • Natural Inhibitors of Coagulation
  • Hemostasis in the Newborn
  • Detection of Hemostatic Defects
  • Common Confirmatory Coagulation Assays
  • Global Hemostatic Tests
  • Preoperative Evaluation of Hemostasis
  • Acquired Coagulation Factor Disorders
  • Vitamin K Deficiency
  • Hepatic Dysfunction
  • Disseminated Intravascular Coagulation
  • Inherited Coagulation Factor Disorders
  • Hemophilia A and B
  • Hemophilia A Carrier Detection
  • Hemophilia B Carrier Detection
  • Prenatal Diagnosis
  • Clinical Course of Hemophilia
  • Treatment (Factor Replacement Therapy)
  • Ancillary Therapy
  • 1-Deamino-8-D-Arginine Vasopressin
  • Antifibrinolytic Therapy
  • Management of Inhibitors in Hemophilia
  • Low Responders
  • High Responders
  • Activated Prothrombin Complex Concentrate (Autoplex and FEIBA)
  • Recombinant Factor VIIa (NovoSeven)
  • Plasmapheresis with Immunoadsorption
  • Immune-Tolerance Induction
  • Treatment of Factor IX Inhibitors
  • Spontaneously Acquired Inhibitory Antibodies to Coagulation Factors
  • Acquired Hemophilia A
  • Acquired Antibodies to Other Coagulation Factors
  • Lupus Anticoagulant
  • von Willebrand Disease
  • Diagnosis and Treatment of vWD
  • Type 1 vWD
  • Type 2A vWD
  • Type 2B vWD
  • Type 2N vWD
  • Type 2M vWD
  • Type 3 vWD
  • Platelet-Type Pseudo-vWD
  • Acquired vWD
  • Rare Coagulation Factor Disorders (FII, V, VII, X, XI, XIII, Fibrinogen Deficiencies)
  • Thrombotic Disorders
  • Mechanisms of Thrombosis in Inherited Thrombophilia
  • Venous Thrombosis
  • Specific Risk Factors for Venous Thrombosis
  • Homocysteine
  • Lipoprotein (a)-Lp (a)
  • Fibrin Degradation Products (d-Dimers)
  • Risk Factors for Recurrence
  • Arterial Thrombosis
  • Arterial Catheterization
  • Cardiac Procedures
  • Blalock-Taussig Shunts
  • Fontan Operation
  • Endovascular Stents
  • Umbilical Artery Catherization
  • Renal Artery Thrombosis
  • Hepatic Artery Thrombosis
  • Kawasaki Disease
  • Antiphospholipid Antibody Syndrome
  • LA and Thrombosis
  • Anticardiolipin Antibodies
  • Treatment
  • Hereditary Thrombotic Disorders
  • Factor V Leiden (Activated Protein C Resistance)
  • Diagnosis
  • Prothrombin G20210A Mutation (FII G20210A)
  • Anti-thrombin Deficiency
  • Treatment
  • 5,10-Methylenetetrahydrofolate Reductase Mutation
  • Protein C Deficiency
  • Treatment
  • Protein S Deficiency
  • Treatment
  • Dysfibrinogenemia
  • Heparin Cofactor II (HC-II) Deficiency
  • Hereditary Defects of Fibrinolytic System
  • Type I Dysplasminogenemia
  • Type II Dysplasminogenemia
  • Tissue Plasminogen Activator Deficiency
  • Plasminogen Activator Inhibitor Deficiency
  • Prophylaxis in Relatives of Patients with Thrombophilia
  • Thrombotic Disorders in Newborns
  • Congenital
  • Acquired
  • Systemic Venous Thromboembolic Disorders
  • Central-Venous-Catheter-Related Thrombosis
  • Umbilical Venous Catheters
  • Central Venous Catheters
  • Renal Vein Thrombosis
  • Systemic Arterial Thromboembolic Disorders
  • Arterial Ischemic Stroke
  • Antithrombotic Agents
  • Heparin Therapy
  • Duration of Heparin Therapy
  • Heparin Antidote
  • Low-Molecular-Weight Heparin
  • Indications for LMWH Therapy
  • Dose (Enoxaparin) (Lovenox, Aventis)
  • Monitoring of LMWH Therapy
  • Duration of LMWH Therapy
  • Adjusting LMWH Dose
  • Antidote for LMWH
  • Heparin-Induced Thrombocytopenia
  • Fondaparinux
  • Argatroban
  • Orgaran (Heparin Sulfate) (Danaproid Sodium)
  • Dabigatran (Pradaxa)
  • Warfarin Therapy
  • Warfarin Daily Loading Doses (Approximately 3-5 Days)
  • Warfarin Maintenance Doses for Long-Term Therapy
  • Antiplatelet Therapy
  • Thrombolytic Therapy
  • Tissue Plasminogen Activator
  • Urokinase
  • Streptokinase
  • Monitoring Response of Thrombolytic Therapy
  • Complications of Thrombolytic Therapy
  • Antithrombotic Therapy in Special Conditions
  • Central Venous Access Devices/Umbilical Venous Catheters
  • Valve Replacement
  • Mechanical Valve Replacement
  • Aortic Valve Replacement
  • Mitral and Tricuspid Valve Replacement
  • Fontan Procedure
  • Blalock-Taussig Shunts
  • Acute Arterial Infarct with Evidence of Dissection in Cerebral or Carotid Arteries
  • Idiopathic Arterial Infarct Without Evidence of Dissection
  • Arterial Infarct with Moyamoya Syndrome
  • Transient Ischemic Attacks
  • Acute Stroke Without Hemorrhage
  • Cerebral Sinovenous Thrombosis
  • Kawasaki Disease
  • Further Reading and References
  • 16 Lymphoproliferative Disorders
  • Lymphoproliferative Disorders
  • Angioimmunoblastic Lymphadenopathy with Dysproteinemia
  • Diagnosis
  • Prognosis
  • Treatment of AILD-Type Lymphoma
  • Small Lymphocytic Infiltrates of the Orbit and Conjunctiva (Ocular Adnexal Lymphoid Proliferation, Pseudolymphoma, Benign L...
  • Angiocentric Immunolymphoproliferative Disorders
  • Clinical Features
  • Lymphomatoid Granulomatosis
  • Midline Lethal Granuloma
  • Postmalignancy Angiocentric Immunolymphoproliferative Lymphoma
  • Treatment
  • Castleman Disease (Angiofollicular Lymph Node Hyperplasia, Benign Giant Lymph Node Hyperplasia, Angiomatous Lymphoid Hamartoma)
  • Clinical Features
  • Unicentric Hyaline Vascular Variant
  • Unicentric Plasma Cell Variant
  • Multicentric Plasma Cell Variant
  • Prognosis
  • Treatment
  • EBV-Associated LPDs in Immunocompromised Individuals
  • EBV Antigens Associated with the Lytic Cycle
  • Cellular Responses in the Control of EBV Infection
  • Reasons for Persistence of Latency in EBV Infection
  • Biological Factors of Significance Involved in the Pathogenesis of LPDs in this Population
  • Iatrogenically Induced Immunodeficiencies
  • Diagnosis of PT-LPDs
  • Treatment
  • Treatment of B-Cell Lymphoproliferative Disease in Immunosuppressed Patients
  • General Treatment
  • Specific Treatment
  • Monoclonal with a Lymphoma-Specific Genetic Abnormality
  • XLP Syndrome
  • Pathophysiology
  • Clinical Manifestations
  • Laboratory Manifestations
  • Treatment
  • Prognosis
  • Autoimmune Lymphoproliferative Syndrome (ALPS) (Canale-Smith Syndrome)
  • Clinical Manifestations
  • Laboratory Manifestations
  • Pathophysiology
  • Diagnostic Criteria
  • Treatment
  • Prognosis
  • Dianzani Autoimmune Lymphoproliferative Disease
  • Lymphomatoid Papulosis in Children
  • Histology
  • Treatment
  • Prognosis
  • Further Reading and References
  • 17 Myelodysplastic Syndromes and Myeloproliferative Disorders
  • Myelodysplastic Syndromes
  • Diagnostic Criteria and Classification
  • Epidemiology
  • Pathophysiology
  • Clinical Features
  • Cytogenetics
  • Molecular Genetics
  • Differential Diagnosis
  • Prognosis
  • Treatment
  • Myeloid Proliferations in Children with DS
  • Incidence
  • Biology
  • Treatment
  • Treatment of TAM in DS
  • Juvenile Myelomonocytic Leukemia
  • Epidemiology
  • Clinical Features
  • Laboratory Features
  • Differential Diagnosis
  • Biology
  • Molecular Genetic Events
  • Natural History
  • Prognosis
  • Treatment
  • Hematopoietic Stem Cell Transplantation
  • Pre-HSCT Approaches
  • Targeted/Investigational Therapies
  • Treatment Options for Patients with Relapse After Allogeneic HSCT
  • Myeloproliferative Neoplasms
  • CML, BCR-ABL1 Positive
  • Incidence
  • Clinical Phases
  • Chronic Phase
  • Signs and Symptoms
  • Laboratory Findings
  • Accelerated Phase
  • Blast Crisis
  • Biology of CML
  • Treatment
  • Treatment of Chronic Phase of CML
  • Mechanism of Action of Imatinib
  • Side Effects of Imatinib
  • Treatment of Advanced Phases of CML
  • Treatment of CML in Accelerated Phase
  • Treatment of CML in Blastic Phase (Blast Crisis)
  • Allogeneic Stem Cell Transplantation for CML
  • Essential Thrombocythemia and Polycythemia Vera
  • Primary Myelofibrosis
  • Clinical Features
  • Hematologic Findings
  • Differential Diagnosis
  • Complications
  • Genetic Mechanisms
  • Treatment
  • Further Reading and References
  • 18 Acute Lymphoblastic Leukemia
  • Incidence of ALL
  • Etiology
  • Clinical Features of ALL
  • General Systemic Effects
  • Hematologic Effects Arising from Bone Marrow Invasion
  • Clinical Manifestations Arising from Lymphoid System Infiltration
  • Clinical Manifestations of Extramedullary Invasion
  • Central Nervous System Involvement
  • Genitourinary Tract Involvement
  • Testicular Involvement
  • Ovarian Involvement
  • Priapism
  • Renal Involvement
  • Gastrointestinal Involvement
  • Bone and Joint Involvement
  • Skin Involvement
  • Cardiac Involvement
  • Lung Involvement
  • Diagnosis
  • Laboratory Studies
  • Classification
  • Morphology
  • Light Microscopy
  • Immunology
  • Immunophenotype Distribution of ALL
  • Acute Leukemia of Ambiguous Lineage
  • Cytogenetics and Molecular Characteristics
  • Molecular Genetics of ALL
  • Future Directions in ALL Classifications
  • Prognostic Factors
  • Treatment
  • Treatment of Newly Diagnosed ALL
  • Treatment of B-Lineage ALL
  • Treatment of Mature B-Cell Lymphoma/Leukemia
  • Treatment of T-Cell ALL
  • Infant Leukemia
  • Philadelphia-Positive ALL
  • Down Syndrome and ALL
  • Relapse in Children with ALL
  • Central Nervous System Relapse
  • Treatment
  • Toxicity of CNS Treatment
  • Testicular Relapse
  • Future Drugs in ALL Therapy
  • Further Reading and References
  • 19 Acute Myeloid Leukemia
  • Incidence and Epidemiology
  • Etiology and Predisposing Conditions
  • Clinical Features
  • Diagnosis
  • Laboratory Studies
  • Blood count and bone marrow
  • Classification of AML
  • Immunophenotype of AML
  • Molecular Genetics of AML
  • Treatment
  • General Care
  • Management of Infectious Complications
  • Transfusions
  • Management of Tumor Lysis Syndrome
  • Management of Hyperleukocytosis
  • Cardioprotection
  • Minimal Residual Disease and Its Implication in the Management of AML
  • Treatment of Newly Diagnosed AML
  • Hematopoietic Stem Cell Transplant for AML
  • Prognosis of Newly Diagnosed AML
  • Relapsed and Refractory AML
  • Novel Therapeutic Approaches
  • Monoclonal Antibodies
  • Tyrosine Kinase Inhibitors
  • Proteasome Inhibitors
  • Other Novel Agents
  • Epigenetics
  • Chimeric Antigen Receptor T-cell Immunotherapy in AML
  • Use of Killer Immunoglobulin Receptors-mismatched Natural Killer Cells in HSCT
  • Acute Promyelocytic Leukemia
  • Background
  • Treatment of APML
  • Supportive Care for Patients with APML
  • Differentiation Syndrome
  • AML Special Subgroups
  • Infant AML
  • Myeloid Leukemia of DS
  • Acute Mixed Lineage Leukemia (Acute Leukemia of Ambiguous Lineage)
  • Further Reading and References
  • 20 Histiocytosis Syndromes
  • Langerhans Cell Histiocytosis
  • Incidence
  • Pathology
  • Pathogenesis
  • Clinical Features
  • Involvement by Site of Disease
  • Skeleton
  • Skin
  • Lungs
  • Liver
  • Hematopoietic System
  • Lymph Nodes
  • Endocrine System
  • Gastrointestinal System
  • Central Nervous System
  • Histopathology of CNS Lesions
  • Hypothalamic Pituitary Involvement
  • Laboratory Studies for the Diagnosis of DI
  • Space-Occupying Central Nervous System Lesions
  • Neurodegenerative Disease
  • Clinical and Laboratory Evaluation of LCH
  • Diagnostic Evaluation
  • Laboratory Testing
  • Routine Blood and Serum Tests
  • Urine Testing
  • Diagnostic Biopsy
  • Radiographic Studies
  • At Diagnosis
  • Follow-Up Radiograph
  • Special Situations
  • Treatment of LCH
  • Specific Site
  • Solitary Bone Lesions
  • Localized Skin Involvement
  • Severe or Refractory Skin Disease
  • Solitary Lymph Node
  • Regional Lymph Node Involvement
  • Meningeal Disease and Brain Parenchyma Disease
  • Multisystem Disease
  • Recurrent or Refractory Disease
  • Prognosis
  • Sequelae and Complications
  • Long-Term Complications
  • Other Histiocytic Disorders
  • Secondary Dendritic Cell Processes
  • Dermal Dendrocyte Disorders
  • Juvenile Xanthogranuloma
  • Erdheim-Chester Disease
  • Solitary Histiocytomas with Dendritic Cell Phenotypes
  • Macrophage Mediated Disorders
  • Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease)
  • Complications
  • Diagnosis
  • Prognosis
  • Treatment
  • Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndromes)
  • Familial or Primary Hemophagocytic Lymphohistiocytosis
  • Pathophysiology, Immunology, and Genetics
  • Clinical Features
  • Treatment
  • Nonfamilial HLH
  • Infection-Associated HLH
  • Treatment
  • Malignancy-Associated Hemophagocytic Syndrome
  • Treatment
  • Macrophage Activation Syndrome in Systemic Juvenile Rheumatoid Arthritis and Other Chronic Conditions (Reactive HLH)
  • Treatment
  • Malignant Histiocytic Disorders in Children
  • Treatment/Outcome
  • Localized Disease
  • Disseminated/Multisystem Disease
  • Recurrent/Refractory Disease
  • Further Reading and References
  • 21 Hodgkin Lymphoma
  • Etiology and Epidemiology
  • Risk Factors
  • Familial Hodgkin Lymphoma
  • EBV-Associated Hodgkin Lymphoma
  • Socioeconomic Status and Hodgkin Lymphoma
  • Biology
  • Pathology
  • Macroscopic Features
  • Histology
  • Immunophenotypic Features
  • Clinical Presentation
  • Constitutional B Symptoms
  • Peripheral Lymphadenopathy
  • Mediastinal Adenopathy
  • Pulmonary
  • Spleen
  • Bone
  • Hematology and Bone Marrow
  • Liver
  • Kidney
  • Nervous System
  • Diagnostic Evaluation and Staging
  • Diagnostic Evaluation
  • Staging
  • Prognostic Factors
  • Treatment
  • Surgery
  • Chemotherapy
  • Radiation
  • Treatment for Refractory or Recurrent Disease
  • Long-Term Complications
  • Secondary Malignancies
  • Cardiac Toxicity
  • Pulmonary Dysfunction
  • Thyroid Dysfunction
  • Gonadal Dysfunction and Infertility
  • Male Gonadal Toxicity
  • Female Gonadal Toxicity
  • Psychosocial and Neurocognitive Impairment
  • Follow-Up Evaluations
  • During Therapy
  • After Completion of Therapy
  • Monitoring for Long-Term Outcomes
  • Further Reading and References
  • Web Resources
  • 22 Non-Hodgkin Lymphoma
  • Introduction
  • Incidence and Epidemiology
  • Incidence
  • Epidemiology
  • Pathologic Classification
  • Clinical Features
  • Diagnosis
  • Staging
  • Prognosis
  • Management
  • Emergency Treatment
  • Chemotherapy
  • Lymphoblastic Lymphoma
  • B Lineage NHL
  • Primary Mediastinal Large B-Cell Lymphoma
  • Anaplastic Large-Cell Lymphoma
  • Rare Pediatric NHL
  • Radiation Therapy
  • Surgical Therapy
  • Management of Relapse
  • Further Reading and References
  • 23 Central Nervous System Malignancies
  • Epidemiology
  • Pathology
  • Supratentorial Lesions
  • Infratentorial Lesions
  • Ventricular Lesions
  • Molecular Pathology of CNS Neoplasms
  • Clinical Manifestations
  • Intracranial Tumors
  • General Signs and Symptoms of Intracranial Tumors
  • Spinal Tumors
  • Genetic Syndromes Associated with Pediatric Brain Tumors
  • Neurofibromatosis Type 1
  • Neurofibromatosis Type 2
  • Li-Fraumeni Syndrome
  • Von Hippel-Lindau
  • Tuberous Sclerosis
  • Turcot Syndrome
  • Gorlin Syndrome
  • Diagnostic Evaluation
  • Computed Tomography
  • Magnetic Resonance Imaging
  • Magnetic Resonance Angiography
  • Magnetic Resonance Spectroscopy
  • Positron Emission Tomography
  • Evaluation of the Spinal Cord
  • Cerebrospinal Fluid Examination
  • Bone Marrow Aspiration and Bone Scan
  • Treatment
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Astrocytomas
  • Management of Astrocytomas
  • Low-Grade Astrocytomas (WHO Grades I and II)
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Recurrence
  • High-Grade Astrocytomas (WHO Grades III and IV)
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Medulloblastoma
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Relapsed Medulloblastoma
  • Molecular Subgroups
  • WNT Tumors
  • Sonic Hedgehog Tumors
  • Group 3 Tumors
  • Group 4 Tumors
  • Brain Stem Tumors
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Ependymomas
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Optic Glioma
  • Surgery
  • Chemotherapy
  • Recommendations
  • Prognosis
  • Craniopharyngiomas
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Intracranial Germ Cell Tumors
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Prognosis
  • Malignant Brain Tumors in Infants and Children Less Than 3 Years of Age
  • Further Reading and References
  • 24 Neuroblastoma
  • Epidemiology
  • Predisposition
  • Pathology and Biology
  • Clinical Features
  • Anatomic Site
  • Other Symptoms
  • Paraneoplastic Syndromes
  • Diagnosis and Staging
  • Staging Systems
  • Treatment
  • Surgery
  • Radiation Therapy
  • Prognosis, Risk Stratification, and Therapy
  • Low-Risk Group
  • Treatment of Low-Risk Patients
  • Patients with INSS Stage 1 and 2 Tumors
  • Patients with INSS Stage 4S Disease
  • Intermediate-Risk Group
  • Treatment of Intermediate-Risk Patients
  • Chemotherapy
  • Surgery
  • Radiation Therapy
  • High-Risk Group
  • Induction Therapy
  • Consolidation Therapy
  • Postconsolidation Continuation Therapy
  • Post-Therapy Monitoring
  • Post-Therapy Surveillance Recommendations
  • Special Treatment Considerations
  • Dumbbell Neuroblastoma and Spinal Cord Compression
  • Opsoclonus Myoclonus Ataxia Syndrome (OMAS)
  • Treatment
  • Neuroblastoma in the Adolescent and Young Adult
  • Further Reading and References
  • 25 Renal Tumors
  • Wilms' Tumor
  • Associated Congenital Anomalies
  • WAGR Syndrome
  • Denys-Drash Syndrome
  • Beckwith-Wiedemann Syndrome
  • Screening of Children with WAGR, Denys-Drash, and Beckwith-Wiedemann Syndromes
  • Signs and Symptoms
  • Diagnostic Studies
  • Staging System
  • Pathology
  • Anaplastic Wilms' Tumor
  • Histologic Findings
  • Loss of Heterozygosity as a Prognostic Factor for Wilms' Tumor
  • Gain of Chromosome 1q as a Prognostic Factor for Wilms' Tumor
  • Treatment
  • Surgery
  • Treatment of Tumors Considered Inoperable
  • Radiation Therapy
  • Bilateral Wilms' Tumor
  • Treatment Approach
  • Post-Therapy Follow-Up
  • Prognosis
  • Treatment in Relapse
  • Standard Risk Relapse
  • High-Risk Relapse
  • Very High Risk of Relapse
  • Local Control for Patients with Relapsed Disease
  • Nephroblastomatosis
  • Congenital Mesoblastic Nephroma
  • Clear Cell Sarcoma of the Kidney
  • Rhabdoid Tumor of the Kidney
  • Renal Cell Carcinoma
  • Further Reading and References
  • 26 Rhabdomyosarcoma and Other Soft-Tissue Sarcomas
  • Incidence and Epidemiology
  • Pathologic and Genetic Classification
  • Genetics of RMS
  • Clinical Features
  • Primary Sites
  • Signs and Symptoms
  • Diagnostic Evaluation
  • Staging
  • Staging for RMS
  • Staging for NRSTS
  • Prognosis
  • Prognosis for RMS
  • Prognosis for NRSTS
  • Treatment
  • Treatment for RMS
  • General Principles
  • Local Control
  • Systemic Control
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Standard Treatment of RMS
  • Treatment Regimens by Risk-Adapted Classification
  • Low-Risk Group
  • Radiation Therapy
  • Chemotherapy
  • Intermediate-Risk Group
  • High-Risk Group
  • Treatment of NRSTS
  • Follow-Up After Completion of Therapy
  • First Year After Completion of Therapy
  • Second and Third Years After Completion of Therapy
  • Fourth and Fifth Years After Completion of Therapy
  • Recurrent Disease
  • Treatment
  • Future Perspectives
  • Further Reading and References
  • 27 Malignant Bone Tumors
  • Osteosarcoma
  • Epidemiology
  • Pathology
  • Clinical Manifestations
  • Diagnostic Evaluation
  • Treatment of Localized Osteosarcoma
  • Surgery
  • Chemotherapy
  • Treatment of Metastatic Osteosarcoma at Presentation
  • Treatment of Relapsed Osteosarcoma
  • Newer Agents Under Investigation
  • Posttreatment Surveillance
  • Prognosis
  • Ewing Sarcoma Family of Tumors
  • Epidemiology
  • Pathology
  • Molecular Genetics
  • Clinical Features
  • Diagnostic Evaluation
  • Treatment
  • Surgery
  • Radiation
  • Chemotherapy
  • Treatment of Metastatic EFT
  • Treatment of Relapsed EFT
  • Prognosis
  • Other Bone Tumors
  • Chondrosarcoma
  • Pathology
  • Clinical Features
  • Diagnostic Evaluation
  • Treatment
  • Posttreatment Surveillance
  • Giant Cell Tumor of Bone
  • Clinical Features
  • Diagnostic Evaluation
  • Treatment
  • RANK-L Inhibition
  • Further Reading and References
  • 28 Retinoblastoma
  • Incidence
  • Classification
  • Laterality
  • Unilateral Tumors
  • Bilateral Tumors
  • Focality
  • Genetics
  • The Two-Hit Hypothesis
  • The RB1 Gene
  • Hereditary RB
  • Nonhereditary RB
  • Genetic Counseling
  • Prenatal Diagnosis and Further Genetic Counseling
  • 13q Deletion Syndrome
  • Risk for Second Malignant Neoplasms
  • Pathology
  • Retinoblastoma
  • Retinocytoma
  • Clinical Features
  • Presenting Signs and Symptoms
  • Differential Diagnosis
  • Pseudoretinoblastoma
  • Patterns of Spread
  • Intraocular
  • Extraocular
  • Trilateral RB
  • Diagnostic Procedures
  • Screening
  • Diagnosis of Intraocular RB
  • Defining Extent of Disease
  • Extraocular Extent of the Disease
  • Classification
  • Treatment
  • Treatment of Intraocular RB
  • Systemic Chemotherapy
  • External Beam Radiotherapy
  • Local Ophthalmic Therapies
  • Cryotherapy
  • Laser Photoablation
  • Transpupillary Thermotherapy
  • Intravitreal Chemotherapy
  • Plaque Radiotherapy
  • Subtenon (Subconjunctival) Chemotherapy
  • Intra-Arterial Chemotherapy
  • Enucleation
  • Treatment of Extraocular RB
  • Treatment of Recurrent RB
  • Posttreatment Management
  • Disease-Related Follow-Up
  • Toxicity-Related Follow-Up
  • Future Perspectives
  • Further Reading and References
  • 29 Germ Cell Tumors
  • Incidence
  • Pathology
  • Clinical Features
  • Diagnostic Evaluation
  • Tumor Markers
  • Staging
  • Treatment
  • Teratoma
  • Mature Teratoma
  • Immature Teratoma
  • Teratoma with Malignant Germ Cell Elements
  • Sacrococcygeal Teratoma
  • Treatment
  • Surgery
  • Chemotherapy
  • Prognosis
  • Ovarian Teratoma
  • Treatment
  • Surgery
  • Chemotherapy
  • Prognosis
  • Mediastinal Teratoma
  • Treatment
  • Surgery
  • Chemotherapy
  • Prognosis
  • GCTs with Malignant Elements
  • Germinoma
  • Ovarian Dysgerminoma
  • Treatment
  • Surgery
  • Chemotherapy
  • Radiotherapy
  • Prognosis
  • Extragonadal Germinoma
  • Treatment
  • Surgery
  • Radiotherapy
  • Chemotherapy
  • Endodermal Sinus Tumor (YST)
  • Testicular YST
  • Prepubertal Males
  • Surgery
  • Prognosis
  • Postpubertal Males
  • Surgery
  • Chemotherapy
  • Ovarian Yolk Sac Tumor
  • Treatment
  • Surgery
  • Chemotherapy
  • Radiotherapy
  • Prognosis
  • Embryonal Carcinoma
  • Testicular Embryonal Carcinoma
  • Treatment
  • Surgery
  • Prognosis
  • Ovarian Embryonal Carcinoma
  • Relapsed and Resistant GCTs
  • Further Reading and References
  • 30 Hepatic Tumors
  • Incidence
  • Epidemiology
  • Pathology
  • Clinical Features
  • Diagnostic Evaluation
  • Staging
  • Treatment
  • Hepatoblastoma
  • Surgery
  • Chemotherapy
  • Radiation
  • Hepatocellular Carcinoma
  • Surgery
  • Chemotherapy
  • Liver Transplantation
  • Follow-up
  • Prognosis
  • Further Reading and References
  • Hepatic Tumors
  • 31 Hematopoietic Stem Cell Transplantation
  • Allogeneic Stem Cell Transplantation
  • Histocompatibility Testing
  • Donor Selection
  • Suggested Strategy for Donor Selection and Prioritization
  • Secondary Donor Prioritization (After HLA Matching Prioritization)
  • HSC Sources, Collection, and Manipulation
  • Bone Marrow
  • Peripheral Blood Stem Cells
  • Umbilical Cord Blood
  • Graft Manipulation Post-collection
  • ABO Incompatibility
  • Graft-Versus-Host Disease
  • Purging of Malignant Cells
  • Medical Evaluation of HSC Donors
  • Pretransplantation Preparative Regimens (Conditioning)
  • Commonly used Preconditioning Regimens
  • Leukemia
  • Non-Hodgkin Lymphoma (NHL)
  • Solid Tumors
  • Severe Aplastic Anemia (SAA)
  • Fanconi Anemia
  • Miscellaneous Conditions
  • Nonmyeloablative (Reduced-Intensity) Regimens
  • Second Transplantation Regimens
  • Engraftment
  • Evidence of Engraftment
  • Causes of Failure to Engraft
  • Complications of HSCT
  • Immunodeficiency
  • Infections
  • Cytomegalovirus (CMV)
  • Interstitial Pneumonitis
  • Pancytopenia
  • Graft-Versus-Host Disease
  • GVHD Prophylaxis
  • Methotrexate (Short Course)
  • Cyclosporine
  • Tacrolimus
  • Sirolimus
  • Mycophenolate Mofetil
  • Methylprednisolone
  • Acute GVHD
  • Treatment
  • Prophylaxis
  • Therapy
  • Chronic GVHD
  • Limited (Usually Involving Only One Organ)
  • Extensive
  • Prognosis
  • Overlap Syndrome
  • Treatment
  • Sinusoidal Obstruction Syndrome (VOD)
  • Clinical Manifestations
  • Predisposing Factors
  • Prophylaxis
  • Treatment
  • Late Sequelae of Stem Cell Transplantation
  • Recent Advances in HSCT
  • Minimal Residual Disease Assessment Pre-HSCT
  • Adoptive Cell Therapy
  • Outcomes
  • Further Reading and References
  • 32 Management of Oncologic Emergencies
  • Metabolic Emergencies
  • Hyperleukocytosis
  • Clinical Features
  • Risk Factors
  • Tumor Lysis Syndrome
  • Diagnostic Criteria for Laboratory TLS and Clinical TLS
  • Prevention and Management of TLS
  • Prevention
  • Active management
  • Management of the Various Metabolic Derangements in TLS
  • Hyperuricemia
  • Hyperkalemia
  • Hyperphosphatemia
  • Hypocalcemia
  • Renal Dysfunction from Tumor Lysis
  • Mechanisms of Renal Dysfunction
  • Indications for Dialysis Include the Following
  • Cardiothoracic Emergencies
  • Superior Vena Cava Syndrome and Superior Mediastinal Syndrome
  • Etiology
  • Clinical Features
  • Management
  • Abdominal Emergencies
  • Typhlitis
  • Etiology
  • Diagnosis
  • Treatment
  • Perirectal Abscess
  • Management
  • Renal Emergencies
  • Oliguria/Anuria
  • Differential Diagnoses
  • Evaluation
  • Therapy
  • Hypertension
  • Definition
  • Etiology
  • Symptoms
  • Treatment
  • Neurologic Emergencies
  • Evaluation and Diagnosis of Neurologic Emergencies
  • Differential Diagnosis
  • Management
  • Spinal Cord Compression
  • Incidence and Etiology
  • Pathophysiology
  • Clinical Presentation
  • Evaluation
  • Treatment
  • Endocrine Emergencies
  • Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH)
  • Etiology
  • Clinical Features
  • Laboratory Features of SIADH
  • Treatment
  • Hypercalcemia of Malignancy
  • Etiology
  • Clinical Features
  • Treatment
  • Adrenal Insufficiency
  • Etiology
  • Clinical Features
  • Treatment
  • Treatment-Associated Emergencies
  • Anaphylaxis and Hypersensitivity to Chemotherapeutic Agents
  • Etiology
  • Presentation
  • Treatment
  • APL Differentiation Syndrome
  • Presentation
  • Treatment
  • Veno-Occlusive Disease
  • Etiology
  • Treatment
  • Further Reading and References
  • 33 Supportive Care of Patients with Cancer
  • Management of Infectious Complications
  • Febrile Neutropenia
  • Management of Febrile Neutropenia
  • Alterations in Initial FN Management
  • Fungal Infection
  • Fever in the Non-Neutropenic Oncology Patient
  • Infection Prophylaxis
  • Antibacterial Prophylaxis
  • Antifungal Prophylaxis
  • Pneumocystis jiroveci Pneumonia Prophylaxis
  • Antiviral Prophylaxis
  • Preexposure Prophylaxis
  • Postexposure Prophylaxis
  • Suppressive Therapy for Viral Infections
  • Hospital Infection Control Practices
  • Anticipatory Guidance
  • Recognition and Management of Nausea and Vomiting
  • Antiemetic Agents
  • Mucositis
  • Prevention and Treatment of Oral Mucositis
  • Pain Management
  • Developmental Issues in Pediatric Pain Management
  • Assessment of Pain
  • Treatment of Pain
  • Step 1 Therapy: Non-Opioid Analgesics
  • Step 2 Therapy: Weak Opioids
  • Step 3 Therapy: Strong Opioids
  • Step 4 Therapy: Adjuvant Modalities
  • Procedural Pain Management in Children with Cancer
  • Neuropathic Cancer Pain
  • Complementary Therapies
  • Nutritional Status of the Oncology Patient
  • Nutrition Assessment
  • Nutrition Intervention
  • Nutrition and Survivorship
  • Utilization of Hematopoietic Growth Factors
  • Granulocyte Colony-Stimulating Factors
  • Erythropoietin
  • Platelet Growth Factors
  • Interleukin-11
  • TPO-Receptor Agonists
  • Management of Acute Radiation Side Effects
  • Hematologic Toxicity
  • CNS Complications
  • Skin Complications
  • Oral Mucositis
  • Dysgeusia
  • Xerostomia
  • GI Complications
  • Pneumonitis
  • Management of CVCs
  • Types of CVCs
  • Peripherally Inserted Central Catheters
  • External Tunneled CVC
  • Implanted Ports
  • CVC Complications
  • Infection
  • Occlusions
  • Thrombotic Occlusion
  • Catheter-Related Thrombosis
  • Catheter Maintenance
  • Posttreatment Immunizations
  • Palliative Care
  • End of Life Care
  • Common Symptoms at the End of Life
  • Treatment of End of Life Symptoms
  • Palliative RT
  • Palliative Sedation Therapy
  • Multidisciplinary Care
  • Further Reading and References
  • 34 Evaluation, Investigations, and Management of Late Effects of Childhood Cancer
  • Musculoskeletal System
  • Surgery
  • Radiation
  • Screening and Management
  • Cardiovascular System
  • Chemotherapy
  • Anthracyclines
  • Cyclophosphamide
  • Radiation
  • Screening and Management
  • Respiratory System
  • Chemotherapy
  • Bleomycin
  • Nitrosourea
  • Radiation
  • Screening and Management
  • Central Nervous System
  • Surgery
  • Chemotherapy
  • Radiation
  • Screening and Management
  • Endocrine System
  • Surgery
  • Chemotherapy
  • Female Gonadal Function
  • Male Gonadal Function
  • Radiation
  • Screening and Management
  • Gonadal Dysfunction
  • Thyroid Dysfunction
  • Anterior Pituitary Dysfunction
  • Growth Hormone Deficiency
  • Luteinizing and Follicle-Stimulating Hormone Deficiency
  • Precocious Puberty
  • Thyroid-Stimulating Hormone Deficiency
  • Adrenocorticotropin Deficiency
  • Hyperprolactinemia
  • Genitourinary System
  • Surgery
  • Chemotherapy
  • Radiation
  • Screening and Management
  • Ocular System
  • Chemotherapy
  • Radiation
  • Screening and Management
  • Auditory System
  • Chemotherapy
  • Radiation
  • Screening and Management
  • Gastrointestinal System
  • Chemotherapy
  • Radiation
  • Transfusions
  • Screening and Management
  • Immunologic System
  • Obesity
  • Second Malignant Neoplasms
  • Chemotherapy
  • Epipodophyllotoxins
  • Anthracyclines
  • Alkylating Agents
  • Radiation
  • Screening and Management
  • Psychosocial Issues
  • Further Reading and References
  • 35 Psychosocial Aspects of Cancer for Children and Their Families
  • Time of Diagnosis
  • Treatment Initiation
  • Illness Stabilization
  • School Reentry
  • Medical Adherence
  • During Relapses and Recurrences
  • Treatment Outcomes: The Unsuccessful Course
  • Termination of Treatment
  • Palliative/Hospice Care
  • Bereavement
  • Treatment Outcomes: The Successful Course
  • End of Treatment
  • Survivorship
  • Conclusion
  • Further Reading and References
  • 36 Blood Banking Principles and Practices
  • Introduction
  • Donor Selection and Collection
  • Component Preparation
  • Whole-Blood-Derived Products
  • Apheresis-Derived Products
  • RBC and Whole-Blood Products
  • Plasma Products
  • Platelet Products
  • Cryoprecipitate
  • Granulocytes
  • Blood Component Modifications
  • Leukoreduction
  • Irradiation
  • Washed Cellular Components
  • Volume Reduction
  • Special Pediatric Population Needs
  • Neonates
  • Patients on Extracorporeal Life Support and Other Critically Ill Patients
  • Hematopoietic Progenitor Cell Transplant Recipients
  • Sickle Cell Disease Patients
  • Special Blood Banking Products
  • Administration
  • RBCs and Whole Blood
  • Plasma Products
  • Platelet Products
  • Cryoprecipitate
  • Granulocytes
  • Neonatal Immunohematological Issues
  • Immunohematology Techniques
  • Summary
  • Further Reading and References
  • Appendix 1: Hematological Reference Values
  • Fetal and Cord Blood Hematologic Value
  • Red Cell Values and Related Serum Values
  • White Cell Values
  • Platelet Values
  • Coagulation Values
  • Bone Marrow Cells
  • Appendix 2: Biological Tumor Markers
  • Index
  • Back Cover

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