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772 Pages·2016·17.965 MB·English
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LANZKOWSKY’S MANUAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY SIXTH EDITION 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. Nopartofthispublicationmaybereproducedortransmittedinanyformorbyanymeans,electronicormechanical,includingphotocopying, recording,oranyinformationstorageandretrievalsystem,withoutpermissioninwritingfromthepublisher.Detailsonhowtoseek permission,furtherinformationaboutthePublisher’spermissionspoliciesandourarrangementswithorganizationssuchastheCopyright ClearanceCenterandtheCopyrightLicensingAgency,canbefoundatourwebsite:www.elsevier.com/permissions. ThisbookandtheindividualcontributionscontainedinitareprotectedundercopyrightbythePublisher(otherthanasmaybenotedherein). Notices Knowledgeandbestpracticeinthisfieldareconstantlychanging.Asnewresearchandexperiencebroadenourunderstanding,changesin researchmethods,professionalpractices,ormedicaltreatmentmaybecomenecessary. Practitionersandresearchersmustalwaysrelyontheirownexperienceandknowledgeinevaluatingandusinganyinformation,methods, compounds,orexperimentsdescribedherein.Inusingsuchinformationormethodstheyshouldbemindfuloftheirownsafetyandthesafety ofothers,includingpartiesforwhomtheyhaveaprofessionalresponsibility. Tothefullestextentofthelaw,neitherthePublishernortheauthors,contributors,oreditors,assumeanyliabilityforanyinjuryand/or damagetopersonsorpropertyasamatterofproductsliability,negligenceorotherwise,orfromanyuseoroperationofanymethods, products,instructions,orideascontainedinthematerialherein. Medicineisanever-changingfield.Standardsafetyprecautionsmustbefollowed,butasnewresearchandclinicalexperiencebroaden ourknowledge,changesintreatmentanddrugtherapymaybecomenecessaryorappropriate.Readersareadvisedtocheckthemost currentproductinformationprovidedbythemanufacturerofeachdrugtobeadministeredtoverifytherecommendeddose,themethod anddurationofadministrations,andcontraindications.Itistheresponsibilityofthetreatingphysician,relyingonexperienceand knowledgeofthepatient,todeterminedosagesandthebesttreatmentforeachindividualpatient.Neitherthepublishernortheauthors assumeanyliabilityforanyinjuryand/ordamagetopersonsorpropertyarisingfromthispublication. BritishLibraryCataloguing-in-PublicationData AcataloguerecordforthisbookisavailablefromtheBritishLibrary. LibraryofCongressCataloging-in-PublicationData AcatalogrecordforthisbookisavailablefromtheLibraryofCongress. 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 List of Contributors Hisham Abdel-Azim, MD, MS Division of Hematology, Teena Bhatla, MD Division of Pediatric Hematology- Oncology and Blood & Marrow Transplantation, Oncology, NYU Langone Medical Center, New York Children’s Hospital Los Angeles, University of UniversitySchoolofMedicine,NewYork,NY Southern California Keck School of Medicine, AcuteLymphoblastic Leukemia Los Angeles, CA Hematopoietic Stem Cell Transplantation Lionel Blanc, PhD Departments of Molecular Medicine and Pediatrics, Hofstra Northwell School of Medicine; Suchitra S. Acharya, MD, MBBS, DCH Division of Center for Oncology and Cell Biology, Laboratory of Pediatric Hematology-Oncology and Stem Cell Developmental Erythropoiesis, The Feinstein Institute for Transplantation, Steven and Alexandra Cohen Children’s MedicalResearch,Manhasset,NY MedicalCenterofNewYork,HofstraNorthwellSchoolof Medicine,NewHydePark,NewYork General Considerations of Hemolytic Diseases, Red Cell Membrane, and EnzymeDefects Disorders of Coagulation Anurag K. Agrawal, MD Pediatric Hematology and Mary Ann Bonilla, MD Pediatric Hematology-Oncology, Oncology, Children’s Hospital and Research Center St. Joseph’s Children’s Hospital, Paterson, NJ; Division of Oakland,Oakland,CA Pediatric Hematology-Oncology, New York Medical College,Valhalla,NY Supportive Care of Patients withCancer Disorders of White BloodCells Robert J. Arceci, MD, PhD(†) Children’s Center for Cancer and Blood Disorders, and Ronald A. Matricaria Institute James B. Bussel, MD Department ofPediatrics, Division of of Molecular Medicine, Phoenix Children’s Hospital, Hematology, Weill Cornell Medical College and Department of Child Health, College of Medicine, NewYork-PresbyterianHospital,NewYork,NY UniversityofArizona,Phoenix,AZ Disorders of Platelets Histiocytosis Syndromes William L. Carroll, MD Division of Pediatric Hematology- Mark P. Atlas, MD Department of Pediatrics, Hofstra Oncology, NYU Langone Medical Center, New York Northwell School of Medicine; Steven and Alexandra UniversitySchoolofMedicine,NewYork,NY CohenChildren’sMedicalCenterofNewYork,NewHyde AcuteLymphoblastic Leukemia Park,NY CentralNervousSystemMalignancies Jeffrey S. Dome, MD, PhD Department of Pediatrics, George Washington University School of Medicine; Rochelle Bagatell, MD Department of Pediatrics, Perelman Divisions of Hematology and Oncology, Children’s Schoolof Medicine, University ofPennsylvania, Children’s NationalHealthSystem,Washington,DC HospitalofPhiladelphia,Philadelphia,PA Renal Tumors Neuroblastoma Nicholas Bernthal, MD Department of Orthopaedic Elizabeth A. Van Dyne, MD Division of Pediatric Surgery, David Geffen School of Medicine, University of Hematology-Oncology, University of California Los California,LosAngeles,SantaMonica,CA AngelesMattel,Children’sHospital,LosAngeles,CA Malignant BoneTumors Malignant Bone Tumors †Deceased xiii xiv LISTOFCONTRIBUTORS Tarek M. Elghetany, MD Departments of Pathology and Mary S. Huang, MD Department of Pediatrics, Harvard Immunology, and Pediatrics, Baylor College of Medicine, Medical School, Massachusetts General Hospital for TexasChildren’sHospital,Houston,TX Children,Boston,MA Myelodysplastic Syndromes and Myeloproliferative Non-Hodgkin Lymphoma Disorders Rachel Kessel, MD Department of Pediatrics, Hofstra Noah Federman, MD Department of Pediatrics and Northwell School of Medicine; Division of Pediatric Orthopaedic Surgery, David Geffen School of Medicine, Hematology-Oncology and Stem Cell Transplantation, University of California; Division of Pediatric Steven and Alexandra Cohen Children’s Medical Center Hematology-Oncology, University of California Los ofNewYork,NewHydePark,NY,USA Angeles Mattel, Children’s Hospital, UCLA’s Jonsson AcuteMyeloid Leukemia ComprehensiveCancerCenter,LosAngeles,CA MalignantBoneTumors Julie I. Krystal, MD, MPH Department of Pediatrics, Hofstra Northwell School of Medicine; Division of Carolyn Fein Levy, MD Department of Pediatrics, Hofstra Pediatric Hematology-Oncology and Stem Cell Northwell School of Medicine; Divisions of Pediatric Transplantation, Department of Pediatrics, Steven and Hematology-Oncology and Stem Cell Transplantation, Alexandra Cohen Children’s Medical Center of New Department of Pediatrics, Steven and Alexandra Cohen York,NewHydePark,NY Children’s Medical CenterofNew York,NewHyde Park, Evaluation, Investigations, and Management of Late NY Effectsof Childhood Cancer Rhabdomyosarcomaand Other Soft-Tissue Sarcomas JanetL.Kwiatkowski,MD,MSCE DivisionofHematology, James Feusner, MD DepartmentofPediatrics,Universityof Children’s Hospital of Philadelphia; Department of California San Francisco Medical Center; Department of Pediatrics,PerelmanSchoolofMedicineattheUniversityof Oncology, Children’s Hospital and Research Center Pennsylvania,Philadelphia,PA Oakland,Oakland,CA Hemoglobinopathies Supportive Care of Patients with Cancer Jonathan D. Fish, MD Division of Pediatric Hematology- Philip. Lanzkowsky, MBChB, MD, ScD (Honoris Causa), Oncology, Hofstra Northwell School of Medicine, Hemp- FRCP, DCH, FAAP Department of Pediatrics, Hofstra stead, New York; Steven and Alexandra Cohen Children’s Northwell School of Medicine, Hempstead, NY; Division MedicalCenterofNewYork,NewHydePark,NY of Pediatric Hematology-Oncology, Department of Pediatrics, Steven and Alexandra Cohen Children’s Evaluation, Investigations, and Management of Late MedicalCenterofNewYork,NewHydePark,NY Effects of Childhood Cancer Classification and Diagnosis of Anemia in Children; Jason L. Freedman, MD, MSCE Division of Oncology, Lymphadenopathy and Diseases of the Spleen; Anemia Children’sHospitalofPhiladelphia,Philadelphia,PA During the Neonatal Period; Iron-Deficiency Anemia; Management of OncologicEmergencies Megaloblastic Anemia Debra L. Friedman, MD, MS Department of Pediatrics, Ann Leahey, MD Department of Pediatrics, Perelman Vanderbilt University School of Medicine; Division of School of Medicine at the University of Pennsylvania, Pediatric Hematology-Oncology, Vanderbilt-Ingram Children’sHospitalofPhiladelphia,Philadelphia,PA CancerCenter,Nashville,TN Retinoblastoma Hodgkin Lymphoma Jeffrey M. Lipton, MD, PhD Department of Pediatrics and Derek R. Hanson, MD Pediatric Neuro-Oncology, Molecular Medicine, Hofstra Northwell School of HackensackUniversityMedicalCenter,JosephM.Sanzari Medicine, Hempstead, NY; Division of Pediatric Children’sHospital,Hackensack,NJ Hematology-Oncology and Stem Cell Transplantation, CentralNervous SystemMalignancies Steven and Alexandra Cohen Children’s Medical Center ofNewYork,NewHydePark,NY;TheFeinsteinInstitute Inga Hofmann, MD Deparmtent of Pediatrics, Harvard forMedicalResearch,Manhasset,NY Medical School, Dana-Farber/Boston Children’s Cancer BoneMarrow Failure and Blood Disorders Center; Department of Hematopathology,BostonChildren’sHospital,Boston,MA Naomi L.C. Luban, MD Edward J. Miller Blood Donor Myelodysplastic Syndromes and Myeloproliferative Center; Division of Laboratory Medicine and Pathology, Disorders Center for Cancer and Blood Disorders, Children’s xv LISTOFCONTRIBUTORS National Medical Center, Sheikh Zayed Campus for Susan R. Rheingold, MD Children’s Hospital of AdvancedChildren’sMedicine;DepartmentsofPediatrics Philadelphia; Division of Oncology, Perelman School and Pathology, George Washington School of Medicine ofMedicineattheUniversityofPennsylvania,Philadelphia, andHealthSciences,Washington,DC PA BloodBanking Principles and Practices Managementof Oncologic Emergencies Indira Sahdev, MD Department of Pediatrics, Hofstra Catherine McGuinn, MD Department of Pediatrics, Northwell School of Medicine; Division of Pediatric Division of Hematology, Weill Cornell Medical College Hematology-Oncology and Stem Cell Transplantation, andNewYorkPresbyterianHospital,NewYork,NY Steven and Alexandra Cohen Children’s Medical Center Disorders of Platelets ofNewYork,NewHydePark,NY Hematopoietic Stem Cell Transplantation Jill S. Menell, MD Pediatric Hematology-Oncology, St. Joseph’s Children’s Hospital, Paterson, NJ; Division of Vijay G. Sankaran, MD, PhD Department of Pediatrics, Pediatric Hematology-Oncology, New York Medical Harvard Medical School, Division of Hematology- College,Valhalla,NY Oncology, Boston Children’s Hospital & Department of Disorders of WhiteBlood Cells Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA Thomas A. Olson, MD Department of Pediatrics, Emory Diagnostic Molecular and Genomic Methodologies for University School of Medicine; Pediatric Hematology- the Hematologist/Oncologist Oncology, Aflac Cancer and Blood Disorders Center, Children’sHealthcareofAtlanta,Atlanta,GA Susmita N. Sarangi, MD, MBBS Division of Pediatric Hematology-Oncology, Steven and Alexandra Cohen Germ Cell Tumors; Hepatic Tumors Children’s Medical Center of New York, New Hyde Park, NY Julie R. Park, MD Department of Pediatrics, University of Disorders of Coagulation Washington School of Medicine; Department of Hematology-Oncology Education, Division of Pediatric Tsewang Tashi, MD Division of Hematology and Hematology-Oncology, Seattle Children’s Hospital, Hematologic Malignacies, University of Utah School of Seattle,Washington,DC Medicine,SaltLakeCity,UT Neuroblastoma Polycythemia Josef T. Prchal, MD Division of Hematology and DavidT.Teachey,MD DepartmentofPediatrics,Perelman Hematologic Malignancies, Department of Internal School of Medicine at the University of Pennsylvania, Medicine at the University of Utah; Huntsman Cancer Children’sHospitalofPhiladelphia,Philadelphia,PA Hospital, Myeloproliferative Disorders Clinic, Salt Lake City, Utah; First Faculty of Medicine, Charles Lymphoproliferative and Myeloproliferative Disorders University,Prague,CzechRepublic Meg Tippy, PsyD Mental Health and Psychosocial Polycythemia Services, Division of Pediatric Hematology-Oncology and Stem Cell Transplantation, Department of Pediatrics, Louis B. Rapkin, MD Department of Pediatrics, Emory Steven and Alexandra Cohen Children’s Medical Center University School of Medicine; Pediatric Hematology- ofNewYork,NewHydePark,NY Oncology, Aflac Cancer and Blood Disorders Center, Psychosocial Aspects of Cancer for Children and Their Children’sHealthcareofAtlanta,Atlanta,GA Families Hepatic Tumors Adrianna Vlachos, MD Department of Pediatrics, Hofstra Arlene Redner, MD Department of Pediatrics, Hofstra Northwell School of Medicine; Division of Pediatric Northwell School of Medicine; Department of Oncology, Hematology-Oncology and Stem Cell Transplantation, Steven and Alexandra Cohen Children’s Medical Center Steven and Alexandra Cohen Children’s Medical Center ofNewYork,NewHydePark,NY,USA ofNewYork,NewHydePark,NY Acute Myeloid Leukemia BoneMarrow Failure xvi LISTOFCONTRIBUTORS Anne B. Warwick, MD, MPH Department of Pediatrics, Lawrence C. Wolfe, MD Department of Pediatrics, Hofstra F. Edward He´bert School of Medicine, Uniformed Northwell School of Medicine; Division of Pediatric ServicesUniversityoftheHealthSciences,Bethesda,MD Hematology-Oncology and Stem Cell Transplantation, Steven and Alexandra Cohen Children’s Medical Center Renal Tumors ofNewYork,NewHydePark,NY HowardWeinstein,MD DepartmentofPediatrics,Harvard Hematologic Manifestations of Systemic Illness; General Medical School; Division of Pediatric Hematology and ConsiderationsofHemolyticDiseases,RedCellMembrane, Oncology, Massachusetts General Hospital for Children, andEnzymeDefects;ExtracorpuscularHemolyticAnemia Boston,MA Non-Hodgkin Lymphoma Edward C.C. Wong, MD Department of Transfusion Medicine,DivisionofLaboratoryMedicine,CenterforCancer Leonard H. Wexler, MD Department of Pediatrics, and Blood Disorders, Children’s National Medical Center Sheikh Zayed Campus for Advanced Children’s Medicine; MemorialSloan-KetteringCancerCenter,NewYork,NY DepartmentofPediatricsandPathology,GeorgeWashington Rhabdomyosarcomaand Other Soft-Tissue Sarcomas SchoolofMedicineandHealthSciences,Washington,DC BloodBanking Principles and Practices About the Editors xvii DRPHILIPLANZKOWSKY

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