Pediatric Hematology

Pediatric Hematology is a comprehensive and succinct referenced text on the diagnosis and treatment of blood diseases in childhood. It provides a ready source of reference for all the conditions likely to be encountered in day-to-day clinical practice. For each condition, the authors give helpful advice on differential diagnoses and clinical management. This third edition has been extensively updated throughout, in light of recent developments in this rapidly advancing area of medicine.A well established textbook on the diagnosis and treatment of blood disorders in childhood A balanced and cutting edge referenced text on all aspects of diagnosis and treatment Easy to use, practically organized, with essential aspects of biology included within each chapter International editorial and contributor team-representing a wide geographical and chronological rangeWhether you are an established sub-specialist in pediatric hematology/oncology, a pediatrician, a trainee or nursing specialist, this book will answer all your questions about benign and malignant disorders of the blood in children and young adults.

Professor Robert Arceci is a world leader in the care of children with leukaemia and especially acute myeloid leukaemia. He edits the main Paediatric Haematology and Oncology journal, ‘Pediatric Blood & Cancer’ and his research is mainly into new agents for leukaemia therapy and the scientific basis for their actions.

Professor Ian Hann is chair of the national NRCN task force on ALL. He is associate medical director of Great Ormond Street Hospital (GOS) and Professor and academic lead in Paediatric Haematology and Oncology at GOS and the Institute of Child Health. He coordinates the current national trial in ALL (ALL trial 2003) and is national coordinator of the international trial in infant leukaemia – Interfant. He is also Editor of the British Journal of Haematology.

Professor Owen Smith is academic lead for Paediatric Haematology and Oncology at the National Children’s Hospital and Trinity College, Dublin. He is a world expert on Shwachmann's syndrome and has carried out world-ranking research on protein C. He is national coordinator for UK and Ireland of the International relapsed AML trial.

List of contributors

ix

Foreword

xii

Preface

xiii

Section 1: The Scientific Basis

Hematopoiesis: an introduction

3

(8)

Hugh J.M. Brady

Section 2: Marrow Failure Syndromes

Failure of red cell production

11

(19)

Sarah E. Ball

Inherited bone marrow failure syndromes

30

(34)

Yigal Dror

Acquired aplastic anemia

64

(15)

Juliana Teo

Yigal Dror

Section 3: Red Cell Disorders

Disorders of iron metabolism: iron deficiency, iron overload and the sideroblastic anemias

79

(26)

Andrew M. Will

Megaloblastic anemia and disorders of cobalamin and folate metabolism

105

(25)

Eric J. Werner

Nonimmune neonatal anemias

130

(21)

Brenda E.S. Gibson

Christina Halsey

Immune hemolytic anemias

151

(20)

Bertil Glader

Disorders of erythrocyte metabolism including porphyria

171

(42)

Lawrence Wolfe

Peter E. Manley

Sickle cell disease

213

(18)

Corrina McMahon

Hemoglobin variants and the rarer hemoglobin disorders

231

(24)

Andreas E. Kulozik

Red cell membrane abnormalities

255

(26)

Patrick G. Gallagher

Thalassemias

281

(24)

Nancy F. Olivieri

David J. Weatherall

Section 4: Granulocyte Disorders

Disorders of granulopoiesis and granulocyte function

305

(35)

Arian Laurence

Pratima Chowdary

Philip Ancliff

Histiocytic disorders

340

(20)

Amir H. Shahlaee

Robert J. Arceci

Acute myeloid leukemia

360

(24)

Leslie S. Kean

Robert J. Arceci

William G. Woods

Chronic myeloid leukemia

384

(21)

Irene A.G. Roberts

Inderjeet S. Dokal

Myelodysplastic syndromes

405

(20)

David K.H. Webb

Section 5: Lymphocyte Disorders

Primary and acquired immunodeficiency

425

(25)

Andrew R. Gennery

Adam H.R. Finn

Andrew J. Cant

Clinical features and therapy of lymphoblastic leukemia

450

(32)

Owen P. Smith

Ian M. Hann

Lymphomas

482

(25)

O.B. Eden

Ross Pinkerton

Section 6: Platelet Disorders

Inherited and congenital thrombocytopenia

507

(19)

Owen P. Smith

Idiopathic thrombocytopenic purpura

526

(22)

Paul Imbach

Thrombocytosis

548

(14)

Christof Dame

Platelet function disorders

562

(23)

Alan D. Michelson

Section 7: Coagulation Disorders

Hemophilia A and B

585

(13)

Judith Smith

Owen P. Smith

von Willebrand disease

598

(10)

David Lillicrap

Rare congenital hemorrhagic disorders

608

(16)

Nigel S. Key

Margaret A. Heisel-Kurth

Acquired disorders of hemostasis

624

(19)

Elizabeth A. Chalmers

Michael D. Williams

Angela Thomas

Bleeding in the neonate

643

(29)

Elizabeth A. Chalmers

Thromboembolic complications in children

672

(21)

Mary Bauman

M. Patricia Massicotte

Section 8: Supportive Therapy

Blood components and fractionated plasma products: preparation, indications and administration

693

(31)

Nancy Robitaille

Heather A. Hume

Hazards of transfusion

724

(21)

Naomi L.C. Luban

Edward C.C. Wong

Management of infection in children with bone marrow failure

745

(12)

Subarna Chakravorty

lan M. Hann

Section 9: Secondary Problems

Hematologic effects of systemic disease and nonhematopoietic tumors

757

(21)

Angela Thomas

Lysosomal storage disorders

778

(14)

Brad T. Tinkle

Gregory A. Grabowski

Reference values

792

(19)

Paula S. Simpkin

Roderick F. Hinchliffe

Index

811

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Summary

Author Biography

Table of Contents

Supplemental Materials

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Pediatric Hematology

Summary

Author Biography

Table of Contents

Supplemental Materials

Digital License