Manual of Pediatric Hematology and Oncology (eBook)

Front Cover 1
Manual of Pediatric Hematology and Oncology 4
Copyright Page 5
Contents 6
Dedication 8
Contributors 10
Introduction 16
Preface to the Fifth Edition 23
Preface to the Fourth Edition 25
Preface to the Third Edition 27
Preface to the Second Edition 29
Preface to the First Edition 30
Chapter 1. Classification and Diagnosis of Anemia in Children 32
Suggested Reading 44
Chapter 2. Anemia During the Neonatal Period 45
Hemorrhage 45
Hemolytic Anemia 50
Failure of Red Cell Production 64
Anemia of Prematurity 64
Physiologic Anemia 66
Diagnostic Approach to Anemia in the Newborn 66
Suggested Reading 68
Chapter 3. Iron-Deficiency Anemia 69
Prevalence 69
Etiologic Factors 69
Non-Hematological Manifestations 75
Diagnosis 75
Treatment 84
Suggested Reading 88
Chapter 4. Megaloblastic Anemia 89
Vitamin B[sub(12)] (Cobalamin) Deficiency 89
Folic Acid Deficiency 104
General Clinical Features of Cobalamin and Folate Deficiency 111
Diagnosis 112
Treatment 115
Suggested Reading 117
Chapter 5. Hematologic Manifestations of Systemic Illness 118
Hematologic Manifestations of Diseases of Various Organs 118
Chronic Illness 127
Lead Intoxication 140
Nutritional Disorders 140
Bone Marrow Infiltration 141
Suggested Reading 153
Chapter 6. Bone Marrow Failure 154
Aplastic Anemia 154
Acquired Aplastic Anemia 157
Congenital Aplastic Anemias 169
Dyskeratosis congenita 178
Congenital Aplastic Anemias of Unknown Inheritance 181
Diamond-Blackfan Anemia (Congenital Pure Red Cell Aplasia) 182
Transient Erythroblastopenia 188
Congenital Dyserythropoietic Anemia (CDA) 189
Sideroblastic Anemias (Mitochondrial Diseases with Bone Marrow Failure Syndromes) 193
Suggested Reading 197
Chapter 7. Red Cell Membrane and Enzyme Defects 199
General Approach to Diagnosis of Hemolytic Anemia 199
Membrane Defects 203
Paroxysmal Nocturnal Hemogloginuria 216
Enzyme Defects 222
Suggested Reading 230
Chapter 8. Hemoglobinopathies 231
Sickle Cell Disease 231
Unstable Hemoglobins 261
Thalassemias 262
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Suggested Reading 276
Chapter 9. Extracorpuscular Hemolytic Anemia 278
Immune Hemolytic Anemia 278
Nonimmune Hemolytic Anemia 285
Suggested Reading 287
Chapter 10. Polycythemia 288
Polycythemia (Erythrocytosis) in the Newborn 288
Polycythemia in Childhood 292
Polycythemia Vera 293
Primary Familial and Congenital Polycythemia 296
Congenital Polycythemia Due to Altered Hypoxia Sensing with Normal P50 297
Prolyl Hydroxylase Domain (PHD) 2 Mutations 299
Congenital Polycythemia Due to Altered Hypoxia Sensing with Decreased P50 299
Other Causes of Polycythemia 300
Diagnostic Approach 300
Suggested Reading 302
Chapter 11. Disorders of White Blood Cells 303
Quantitative Disorders of Leukocytes 303
Decreased Polymorphonuclear Leukocyte Production 309
Increased Destruction or Disorders of Distribution of Polymorphonuclear Leukocytes 320
Eosinophils 326
Lymphocytes 333
Neutrophil Production and Destruction in Newborn Infants 349
Suggested Reading 350
Chapter 12. Disorders of Platelets 352
Thrombocytopenia in the Newborn 352
Inherited Thrombocytopenias 366
Thrombocytopenia in Childhood 374
Thrombocytosis 390
Qualitative Platelet Disorders 393
Acquired Platelet Disorders 400
Inherited Vascular and Connective Tissue Disorders 403
Laboratory Evaluation of Platelets and Platelet Function 404
Nonthrombocytopenic Purpura 406
Suggested Reading 407
Chapter 13. Hemostatic Disorders 409
Physiology of Hemostasis 409
Fibrinolysis 413
Natural Inhibitors of Coagulation 414
Hemostasis in the Newborn 416
Detection of Hemostatic Defects 418
Acquired Coagulation Factor Disorders 421
Inherited Coagulation Factor Disorders 426
Rare Inherited Coagulation Factor Disorders (Factor II, V, VII, X, XIII, Fibrinogen Deficiencies) 447
Hereditary Disorders of Platelet Function 448
Suggested Reading 449
Chapter 14. Thrombotic Disorders 450
Mechanisms of Thrombosis in Inherited Thrombophilia 450
Venous Thrombosis 450
Arterial Thrombosis 452
Antiphospholipid Syndrome 461
Hereditary Thrombotic Disorders 467
Thrombotic Disorders in Newborns 474
Antithrombotic Agents 476
Heparin-induced Thrombocytopenia 481
Thrombolytic Therapy 486
Antithrombotic Therapy in Special Conditions 489
Suggested Reading 491
Chapter 15. Lymphadenopathy and Splenomegaly 492
Lymphadenopathy 492
Splenomegaly 499
Suggested Reading 502
Chapter 16. Lymphoproliferative Disorders, Myelodysplastic Syndromes and Myeloproliferative Disorders 503
Lymphoproliferative Disorders 503
Myelodysplastic Syndrome 525
Juvenile Myelomonocytic Leukemia (JMML) 534
Chronic Myeloproliferative Diseases 539
Chronic Myeloid Leukemia (CML) 539
Suggested Reading 547
Chapter 17. Leukemias 549
Incidence 549
Etiology 549
Clinical Features of Acute Lymphoblastic Leukemia (ALL) 550
Acute Myeloid Leukemia 581
Suggested Reading 595
Chapter 18. Histiocytosis Syndromes 598
Langerhans Cell Histiocytosis 598
Other Histiocytic Disorders 616
Dermal Dendrocyte Disorders 616
Suggested Reading 627
Chapter 19. Hodgkin Lymphoma 630
Etiology and Epidemiology 630
Risk Factors 630
Biology 631
Pathology 631
Clinical Presentation 634
Diagnostic Evaluation and Staging 638
Prognostic Factors 640
Treatment 642
Long-term Complications 648
Follow-up Evaluations 652
Suggested Reading 653
Chapter 20. Non-Hodgkin Lymphoma 655
Introduction 655
Incidence and Epidemiology 655
Pathologic Classification 656
Clinical Features 656
Diagnosis 661
Staging 661
Management 662
Lymphoblastic Lymphoma 663
B lineage NHL (BC, BLL and DLCL) 664
Suggested Reading 676
Chapter 21. Central Nervous System Malignancies 678
Pathology 678
Clinical Manifestations 680
Diagnostic Evaluation 682
Treatment 685
Astrocytomas 688
Medulloblastoma 691
Brain Stem Tumors 694
Ependymomas 695
Optic Glioma 696
Craniopharyngiomas 698
Intracranial Germ Cell Tumors 699
Malignant Brain Tumors in Infants and Children Less than 3 Years of Age 700
Suggested Reading 701
Chapter 22. Neuroblastoma 702
Epidemiology 702
Predisposition 702
Pathology and Biology 702
Clinical Features 703
Neuroblastoma in the Neonate 706
Diagnosis and Staging 706
Prognosis and Risk Stratification 708
Treatment 710
Disease Stratification and Therapy 712
Post-Therapy Monitoring 721
Special Treatment Considerations 723
Neuroblastoma in the Adolescent and Young Adult 724
Suggested Reading 724
Chapter 23. Renal Tumors 726
Wilms' Tumor 726
Nephroblastomatosis 742
Congenital Mesoblastic Nephroma 742
Clear Cell Sarcoma of the Kidney 743
Rhabdoid Tumor of the Kidney 744
Renal Cell Carcinoma (RCC) 744
Suggested Reading 744
Chapter 24. Rhabdomyosarcoma and Other Soft-Tissue Sarcomas 746
Incidence and Epidemiology 746
Pathologic Classification 749
Genetics 749
Clinical Features 750
Diagnostic Evaluation 752
Staging 753
Prognosis 756
Treatment 758
Follow-Up After Completion of Therapy 765
Recurrent Disease 766
Future Perspectives 767
Suggested Reading 768
Chapter 25. Malignant Bone Tumors 770
Osteosarcoma 770
Ewing Sarcoma 780
Suggested Reading 788
Chapter 26. Retinoblastoma 790
Incidence 790
Classification 790
Other Epidemiologic Data 793
Risk for Second Malignant Neoplasms (SMN) 794
Pathology 794
Clinical Features 795
Diagnostic Procedures 796
Staging 797
Treatment 799
Post-Treatment Management 804
Future Perspectives 805
Suggested Reading 806
Chapter 27. Germ Cell Tumors 807
Incidence 807
Pathology 807
Clinical Features 808
Diagnostic Evaluation 812
Tumor Markers 813
Staging 814
Treatment 815
Relapsed and Resistant Germ Cell Tumors 824
Suggested Reading 825
Chapter 28. Hepatic Tumors 827
Incidence 827
Epidemiology 827
Pathology 828
Clinical Features 829
Diagnostic Evaluation 829
Staging 832
Treatment 832
Prognosis 835
Suggested Reading 836
Chapter 29. Hematopoietic Stem Cell Transplantation 837
Solid Tumors 840
Allogeneic Stem Cell Transplantation 841
Engraftment 850
Graft Versus Host Disease (GVHD) 851
Autologous Stem Cell Transplantation 852
Complications of Hematopoietic Stem Cell Transplantation 854
Late Sequelae of Stem Cell Transplantation 866
Post-Stem Cell Transplantation Therapy 866
Suggested Reading 868
Chapter 30. Management of Oncologic Emergencies 870
Metabolic and Endocrine Emergencies 870
Oncologic Emergencies by Anatomic Region 879
Suggested Reading 887
Chapter 31. Supportive Care of Patients with Cancer 888
Management of Infectious Complications 888
Bacterial Infections 897
Fungal Infections 899
Viral Infections 905
Protozoan Infections 906
G-CSF and Granulocyte Infusions 907
Immunizations 907
Blood Component Therapy 909
Hematopoietic Growth Factors: Basic Biology of Growth Factors 917
Clinical use of G-CSF, GM-CSF, Erythropoietin and IL-11 919
Prevention of Organ Toxicity 923
Pain Management 927
General Guidelines for Management of Pain in Oncology Patients 933
Management of Nausea and Vomiting 935
Nutritional Support 944
Psychosocial Support and End-of-Life Care 949
Suggested Reading 949
Chapter 32. Evaluation, Investigations and Management of Late Effects of Childhood Cancer 952
Musculoskeletal Dysfunction 953
Cardiovascular Disorders 959
Respiratory System 961
Central Nervous System 964
Endocrine System 967
Genitourinary System 973
Ocular System 975
Auditory System 976
Gastrointestinal System 977
Immunologic System 977
Obesity 978
Second Malignant Neoplasms 978
Suggested Reading 981
Chapter 33. Psychosocial Aspects of Cancer for Children and their Families 983
Diagnosis 983
Treatment Initiation 989
Illness Stabilization 990
Relapses and Recurrences 991
Treatment Outcomes: The Unsuccessful Course 992
Treatment Outcomes: The Successful Course 995
Survivorship 995
Conclusion 997
Suggested Reading 997
Appendix 1 Hematological Reference Values 1000
Red Cell Values and Related Serum Values 1000
White Cell Values 1012
Platelet Values 1013
Coagulation Values 1014
Bone Marrow Cells 1022
Appendix 2 Biological Tumor Markers 1026
Appendix 3 Pharmacologic Properties of Commonly used Anticancer Drugs 1028
Index 1036
A 1036
B 1038
C 1039
D 1041
E 1042
F 1043
G 1043
H 1044
I 1047
J 1048
K 1048
L 1048
M 1049
N 1050
O 1051
P 1052
Q 1053
R 1053
S 1054
T 1055
U 1057
V 1057
W 1057
X 1058
Y 1058