Lanzkowsky’s manual of pediatric hematology and oncology PDF
Preview Lanzkowsky’s manual of pediatric hematology and oncology
LANZKOWSKY’S MANUAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY SIXTH EDITION This pageintentionallyleftblank LANZKOWSKY’S MANUAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY SIXTH EDITION Edited by P L , MBC B, MD, S D (H C ), FRCP, DCH, FAAP HILIP ANZKOWSKY H C ONORIS AUSA ChiefEmeritus,PediatricHematology-Oncology ChairmanEmeritus,DepartmentofPediatrics ExecutiveDirectorandChief-of-Staff(Retired) StevenandAlexandraCohenChildren’sMedicalCenterofNewYork,NewHydePark,NewYork VicePresident,Children’sHealthNetwork(Retired) NorthwellHealthSystem ProfessorofPediatrics HofstraNorthwellSchoolofMedicine,Hempstead,NewYork J M. L , MD, P D EFFREY IPTON H ChiefofHematology-OncologyandStemCellTransplantation StevenandAlexandraCohenChildren’sMedicalCenterofNewYork,NewHydePark,NewYork CenterHead,PatientOrientedResearch,FeinsteinInstituteforMedicalResearch,Manhasset,NewYork ProfessorofPediatricsandMolecularMedicine HofstraNorthwellSchoolofMedicine,Hempstead,NewYork Professor,ElmezziGraduateSchoolofMedicine,Manhasset,NewYork J D. F , MD ONATHAN ISH DirectorSurvivorsFacingForwardProgram StevenandAlexandraCohenChildren’sMedicalCenterofNewYork,NewHydePark,NewYork AssistantProfessorofPediatrics HofstraNorthwellSchoolofMedicine,Hempstead,NewYork AMSTERDAM(cid:129)BOSTON(cid:129)HEIDELBERG(cid:129)LONDON NEWYORK(cid:129)OXFORD(cid:129)PARIS(cid:129)SANDIEGO SANFRANCISCO(cid:129)SINGAPORE(cid:129)SYDNEY(cid:129)TOKYO AcademicPressisanimprintofElsevier AcademicPressisanimprintofElsevier 125LondonWall,LondonEC2Y5AS,UK 525BStreet,Suite1800,SanDiego,CA92101-4495,USA 50HampshireStreet,5thFloor,Cambridge,MA02139,USA TheBoulevard,LangfordLane,Kidlington,OxfordOX51GB,UK Copyrightr1989,1994,1999,2005,2011,2016ElsevierInc.Allrightsreserved. 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ISBN:978-0-12-801368-7 ForInformationonallAcademicPresspublications visitourwebsiteathttp://www.elsevier.com/ Publisher:MicaHaley AcquisitionEditor:TariBroderick EditorialProjectManager:JeffreyL.Rossetti ProductionProjectManager:Luc´ıaPe´rez Designer:MarkRogers TypesetbyMPSLimited,Chennai,India Dedication This book is dedicated Toour parents, Abeand Lily Lanzkowsky, Thelma andAl Lipton, andVicky andLawrence Fish, who instilled inus the importance of integrity, the rewards of industry andthe primacy of being a mensch. To ourwives, Rhona Lanzkowsky, Linda Lipton,and Leah Fish, who understand that the study of medicine is a lifelong and consumingprocess. To our childrenand grandchildren, our pride and joy. And To our patients, students,pediatric house staff, fellows in pediatrichematology andoncology, and to our colleagues who have taught usso much over the years. Today hecan discover the errorsof yesterday andtomorrow he may obtainnewlight onwhathe thinks himself sure of today Moses Maimo´nides This page intentionallyleftblank Contents List of Contributors.....................................xiii 3. Classification and Diagnosis About the Editors ....................................... xvii of Anemia in Children....................... 32 Preface to the Sixth Edition.......................... xxi PHILIPLANZKOWSKY Preface to the Fifth Edition........................ xxiii Classification and Diagnosis...............................32 Preface to the Fourth Edition.......................xxv Further Reading and References.........................41 Preface to the Third Edition.......................xxvii Preface to the Second Edition......................xxix 4. Lymphadenopathy and Diseases Preface to the First Edition..........................xxxi of the Spleen....................................... 42 Introduction: Historic Perspective.............xxxiii PHILIPLANZKOWSKY Lymphadenopathy...............................................42 1. Diagnostic Molecular and Genomic Diseases of the Spleen.........................................47 Methodologies for the Splenomegaly.......................................................48 Hematologist/Oncologist...................... 1 Further Reading and References.........................50 VIJAYG.SANKARAN 5. Anemia During the Neonatal Clinical Molecular and Genomic Period.................................................. 51 Methodologies...................................................1 PHILIPLANZKOWSKY Interpreting and Evaluating the Results from Clinical Genetic Testing..........................5 Hemorrhage.........................................................51 Further Reading and References...........................6 Hemolytic Anemia..............................................55 Failure of Red Cell Production...........................65 Anemia of Prematurity........................................65 2. Hematologic Manifestations of Physiologic Anemia.............................................66 Systemic Illness..................................... 7 Diagnostic Approach to Anemia in the Newborn..........................................................66 LAWRENCEC.WOLFE Further Reading and References.........................68 Hematologic Manifestations of Diseases Related to Various Organs................................7 6. Iron-Deficiency Anemia..................... 69 Chronic Illness.....................................................13 PHILIPLANZKOWSKY Lead Intoxication................................................23 Nutritional Disorders...........................................23 Prevalence............................................................69 Bone Marrow Infiltration....................................23 Etiologic Factors..................................................69 Further Reading and References.........................31 Clinical Features..................................................73 vii viii CONTENTS Diagnosis..............................................................74 Membrane Defects.............................................137 Stages of Iron Depletion.....................................78 Paroxysmal Nocturnal Hemoglobinuria...........147 Differential Diagnosis..........................................79 Enzyme Defects..................................................151 Treatment.............................................................81 Further Reading and References.......................157 Further Reading and References.........................83 10. Extracorpuscular Hemolytic 7. Megaloblastic Anemia........................ 84 Anemia........................................... 159 PHILIPLANZKOWSKY LAWRENCEC.WOLFE Vitamin B (Cobalamin) Deficiency.................84 12 Immune Hemolytic Anemia.............................159 Folic Acid Deficiency..........................................93 Clinical Features of Cobalamin NonImmune Hemolytic Anemia......................164 Further Reading and References.......................165 and Folate Deficiency......................................97 Diagnosis of Cobalamin and Folate Deficiency........................................................98 11. Hemoglobinopathies....................... 166 Treatment...........................................................100 JANETL.KWIATKOWSKI Further Reading and References.......................101 Sickle Cell Disease............................................166 8. Bone Marrow Failure........................ 102 Thalassemias......................................................186 ADRIANNAVLACHOSANDJEFFREYM.LIPTON Management of the Acutely Ill Thalassemic Patient............................................................193 Aplastic Anemia................................................102 Further Reading and References.......................195 Acquired Aplastic Anemia...............................102 Inherited Bone Marrow Failure Syndromes .....112 Fanconi Anemia................................................113 12. Polycythemia .................................. 197 Dyskeratosis Congenita.....................................119 TSEWANGTASHIANDJOSEFT.PRCHAL Congenital Aplastic Anemias of Unknown Polycythemia (Erythrocytosis) in the Inheritance.....................................................120 Newborn........................................................197 Diamond(cid:1)Blackfan Anemia (Congenital Polycythemia in Childhood..............................200 Pure Red Cell Aplasia).................................121 Polycythemia Vera.............................................201 Transient Erythroblastopenia of Childhood.....125 Primary Familial and Congenital Congenital Dyserythropoietic Anemia.............126 Polycythemia.................................................203 Sideroblastic Anemias (Mitochondrial Congenital Polycythemia Due to Altered Diseases with Bone Marrow Failure Hypoxia Sensing with Normal P ...............203 Syndromes)....................................................127 50 Congenital Polycythemia Due to Altered Severe Congenital Neutropenia and Hypoxia Sensing with Decreased P ...........204 Kostmann Syndrome.....................................130 50 Other Causes of Polycythemia..........................205 Reticular Dysgenesis..........................................132 Diagnostic Approach.........................................206 Further Reading and References.......................132 Further Reading and References.......................207 9. General Considerations of Hemolytic Diseases, Red Cell Membrane, and 13. Disorders of White Blood Cells..... 209 Enzyme Defects................................. 134 MARYANNBONILLAANDJILLS.MENELL LIONELBLANCANDLAWRENCEC.WOLFE Quantitative Disorders of Leukocytes...............209 Clinical Features of Hemolytic Disease............134 Decreased Production or Intrinsic Laboratory Findings...........................................134 Defects of Neutrophils...................................212 ix CONTENTS Increased Destruction or Extrinsic Thrombotic Disorders in Newborns.................320 Defects of Neutrophils...................................218 Antithrombotic Agents.....................................321 Immune Neutropenia........................................218 Antithrombotic Therapy in Special NonImmune Neutropenia.................................220 Conditions.....................................................330 Eosinophils.........................................................222 Further Reading and References.......................332 Lymphocytes......................................................228 Disorders of Leukocyte Function......................229 16. Lymphoproliferative Disorders....... 334 Disorders of Adhesion and Chemotaxis...........230 DAVIDT.TEACHEY Disorders of Neutrophil Granules.....................232 Lymphoproliferative Disorders..........................334 Disorders of Oxidative Metabolism..................234 Further Reading and References.......................347 Neutrophil Production and Destruction in Newborn Infants............................................237 17. Myelodysplastic Syndromes and Neutrophil Function in the Newborn..............237 Myeloproliferative Disorders.......... 348 Further Reading and References.......................238 INGAHOFMANNANDTAREKM.ELGHETANY Myelodysplastic Syndromes...............................348 14. Disorders of Platelets...................... 239 Juvenile Myelomonocytic Leukemia ................355 CATHERINEMCGUINNANDJAMESB.BUSSEL Myeloproliferative Neoplasms...........................359 Thrombocytopenia in the Newborn.................239 CML, BCR(cid:1)ABL1 Positive..............................360 Inherited Thrombocytopenias...........................249 Essential Thrombocythemia and Immune Thrombocytopenic Purpura (ITP).....254 Polycythemia Vera.........................................364 Other Causes of Thrombocytopenia.................261 Primary Myelofibrosis........................................364 Thrombocytosis.................................................265 Further Reading and References.......................365 Qualitative Platelet Disorders...........................267 Acquired Platelet Disorders..............................273 18. Acute Lymphoblastic Leukemia..... 367 Nonthrombocytopenic Purpura........................277 WILLIAML.CARROLLANDTEENABHATLA Further Reading and References.......................277 Incidence of ALL..............................................367 Etiology..............................................................367 15. Disorders of Coagulation................ 279 Clinical Features of ALL...................................368 SUCHITRAS.ACHARYAANDSUSMITAN.SARANGI Prognostic Factors..............................................376 Treatment...........................................................377 Hemostatic Disorders........................................279 Infant Leukemia................................................379 Hemostasis in the Newborn..............................282 Philadelphia-Positive ALL................................380 Detection of Hemostatic Defects......................284 Down Syndrome and ALL................................383 Acquired Coagulation Factor Disorders...........288 Relapse in Children with ALL.........................383 Inherited Coagulation Factor Disorders...........290 Future Drugs in ALLTherapy...........................387 von Willebrand Disease ....................................301 Further Reading and References.......................388 Rare Coagulation Factor Disorders (FII, V, VII, X, XI, XIII, Fibrinogen Deficiencies)....305 19. Acute Myeloid Leukemia............... 390 Thrombotic Disorders........................................306 ARLENEREDNERANDRACHELKESSEL Venous Thrombosis...........................................308 Arterial Thrombosis..........................................311 Incidence and Epidemiology.............................390 Antiphospholipid Antibody Syndrome............313 Etiology and Predisposing Conditions..............390 Hereditary Thrombotic Disorders.....................316 Clinical Features................................................391
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