Contents
1. Interpretation of the Complete Blood Count 1
Vikas Dua, Vinita Jain, SP Yadav,
Anupam Sachdeva
Red Cell Distribution Width 1
Hematocrit 1
Rule of 3s 2
Blood Smear 2
Role of MCV, RDW and Reticulocyte Count
in Evaluating Anemia 3
Discriminant Function 6
Newborn Infants 7
Platelets 9
White Blood Cells 10
Target Cells 11
Spherocytes 11
Acanthocytes 11
Echinocytes 12
Schistocytes 12
Basophilic Stippling (Aggregated Ribosomes) 12
Howell-Jolly Bodies 13
Siderocytes (Nonhemoglobin Iron) 13
Cabot’s Rings (Nuclear Remnants) 13
Polychromasia 13
Nucleated RBCs 13
Elliptocytes 13
Spiculated/Crenated Cells 13
Bizarre Poikilocytes 13
2. Clinical Approach to a Child with Anemia 15
Nitin K Shah, Himani Manchanda, MR Lokeshwar
Approach to a Child with Anemia 15
Is the Patient Anemic? 15
Definition 15
Clinical Judgment of Anemia 15
How Severe is Anemia? 15
What is the Type and Cause of Anemia? 16
3. Laboratory Evaluation of a Patient
with Anemia 21
Ushma Singh, Shirish Kumar, SP Yadav,
Anupam Sachdeva
Mechanisms and Pathophysiology of Anemia 21
Signs of Accelerated Erythropoiesis 28
Signs of Accelerated RBC Destruction 29
Investigations for Hemolytic Anemia 29
4. Anemia of Prematurity 31
Himani Manchanda, Sachin Thakur,
Dhiren Gupta, Anupam Sachdeva
Pathophysiology 31
Signs and Symptoms 31
Evaluation 31
Differential Diagnosis 32
Treatment 32
5. Anemia in the Newborn 34
Neha Rastogi, Anupam Sachdeva,
SP Yadav, Arun Soni
Fetal Erythropoiesis 34
Normal Hematological Values in
the Neonatal Period 34
Etiology 36
Diagnostic Approach to Anemia in
a Newborn 38
Treatment of Anemia in Newborn 38
6. Nutritional Anemia 41
Neha Rastogi, Anupam Sachdeva,
SP Yadav, Panna Choudhury
Prevalence 41
Iron Deficiency Anemia 41
Iron Metabolism 42
Clinical Features of IDA 43
Consequences of Iron Deficiency 44
Assessment of Iron Status and
Screening for Anemia 44
Treatment 46
Prevention of Nutritional Anemia 47
Macrocytic-Megaloblastic Anemia 49
7. National Nutritional Anemia
Control Program 53
Neha Rastogi, Panna Choudhury,
Anupam Sachdeva
National Nutritional Anemia Prophylaxis
Program 53
Evaluation of Iron Supplementation
Program 53
National Nutritional Anemia Control
Program 53
8. Aplastic Anemia: Current Issues in
Diagnosis and Management 57
Bharat R Agarwal, Nivedita Dhingra
Pancytopenia in Children 57
Immune Physiology of AA 57
Diagnosis of AA 57
Etiology of Aplastic Anemia:
Recognized Causes 59
Management of Aplastic Anemia 63
xx Practical Pediatric Hematology
Definitive Therapy 64
Appendix 1: Practical Issues with
Immunosuppressive Therapy in
Aplastic Anemia 66
9. Approach to the Diagnosis of
Hemolytic Disorders 69
Mohammed Ramzan, SP Yadav,
Anupam Sachdeva
History and Physical Examination 69
Specific Tests to Establish the Cause of
Hemolysis 71
Salient Laboratory Features of Common
Disorders Associated with Hemolytic
Anemia 71
10. Management of -Thalassemia Major 75
Mohammed Ramzan, SP Yadav,
Anupam Sachdeva
Transfusion Therapy 75
Splenectomy 78
Folic Acid 78
Chelation Therapy 78
Iron Chelators 78
Deferiprone (Ferripox, Kelfer, L1) Therapy 80
Newer Chelating Agents 81
Bone Marrow Transplantation 82
Cord Blood Transplantation 82
Alternative Therapies in Thalassemia 83
Gene Therapy 83
11. Hemolytic Anemia (Other than
Thalassemia) Management 85
MB Agarwal
Hereditary Hemolytic Anemias
(Intracorpuscular Defect) 85
Acquired Hemolytic Anemias 87
Chemical Agents 89
Physical Agents 89
Paroxysmal Nocturnal Hemoglobinuria 89
12. Thrombocytosis 90
Vikas Dua, SP Yadav, Vinita Jain,
Anupam Sachdeva
Normal Upper Thrombocyte Levels in
Childhood 90
Physiology of Platelet Production 90
Classification 90
Essential Thrombocytosis 90
Clinical Manifestations 91
Treatment Options 91
Reactive Thrombocytosis 92
Tissue Damage 93
Hypoxia 93
Autoimmune Disease 94
Gastrointestinal Disease without Infection 94
Renal Disease 94
Oncologic Disease 94
Medications 94
Other Causes 94
Complications 94
Indications for Prophylaxis 95
Primary Familial Thrombocytosis 95
13. Hemostasis: Developmental Aspects and
Rare Congenital Bleeding Disorders 97
Nivedita Dhingra, SP Yadav, Anupam Sachdeva
Neonatal Hemostasis 97
Intrinsic Pathway 99
Extrinsic Pathway 100
Common Pathway 100
Protein C/Protein S Anticoagulant Pathway 100
Thrombomodulin 100
Antithrombin 100
Fibrinolysis Pathway 100
Causes of Neonatal Bleeding
(Deranged Hemostasis) 101
Hemophilias A and B 101
Clinical Features 102
von Willebrand Disease 102
Deficiencies of the Intrinsic Pathway Contact
Activating System 102
Factor XI Deficiency 103
Factor X (Stuart-Prower Factor) Deficiency 104
Factor V Deficiency 105
Factor VII Deficiency 106
Deficiencies of the Common Pathway
Prothrombin Deficiency 106
Fibrinogen Deficiency 107
Factor XIII Deficiency 109
Congenital Factor XIII Deficiency 110
Acquired Coagulation Disorders 111
Vitamin K Deficiency Bleeding 112
14. von Willebrand’s Disease 115
Neha Rastogi, Vinita Jain, SP Yadav,
Anupam Sachdeva
Clinical Presentation 115
Laboratory Diagnosis and Monitoring 115
von Willebrand's Disease Classification 117
15. Management of Hemophilia 120
Vikram Mathews
Products for Replacement Therapy 120
Nonfactor Pharmacological Agents 121
Management of Acute Bleeds 121
Management of Chronic Complications 122
Physical Therapy and Rehabilitation 122
Surgical Management 123
Management of Patients with Inhibitors 123
Carrier Detection and Antenatal Diagnosis 124
Recent Advances 124
16. Thrombocytopenia Other than ITP and
Thrombasthenia 125
Sunil Gomber
Clinical Presentation 125
Contributors xxi
Drug-Induced Thrombocytopenia 125
Decreased or Absent Megakaryocytes
in the Marrow 127
17. Immune Thrombocytopenia:
Diagnosis and Management 130
Vasant Chinnabhandar, MR Lokeshwar,
Trupti Dongre
What are Various Types of ITP? 130
Pathophysiology of ITP 131
Diagnosis of ITP 131
Important Factors in Diagnosis 131
Other Investigations 134
Evaluation of Patients with Persistent/
Early Refractory ITP 134
Management of ITP 134
Specific Therapy in ITP 137
Intravenous Immunoglobulin 138
Anti-D in ITP 138
Rituximab 139
Thrombopoietin-Receptor Agonists:
Romiplostim and Eltrombopag 139
Dapsone (Diamino-Diphenyl Sulfone) 140
Splenectomy in ITP 140
Splenectomy in Chronic ITP 140
Are Immunosuppressant Drugs Useful in
Chronic ITP? 141
18. Vitamin K Deficiency Bleeding 144
Jagdish Chandra
Definition and Inclusion Criteria 144
Incidence 145
Clinical Features 145
Diagnosis 145
Treatment 146
Prognosis 146
Prevention 146
19. Neonatal Thrombocytopenia 148
Nivedita Dhingra, Sachin Thakur,
Anupam Sachdeva
Neonatal Autoimmune Thrombocytopenia 150
Nonimmune Thrombocytopenia 151
Hereditary Thrombocytopenia 151
20. Hypercoagulable Disease in Children
and its Diagnostic Approach 153
R Saxena
Acquired Hypercoagulable State 153
Inherited Hypercoagulable States 153
21. Antithrombotic Therapy in Pediatrics 156
ATK Rau, Shruti Patil, Anupam Sachdeva
Unfractionated Heparin 156
Mechanism of Action and Pharmacology 156
Dosing and Administration 157
Prophylactic Therapy 157
Monitoring 157
Adverse Effects, Reversal of Action and
Antidote of UHF 157
Heparin-induced Thrombocytopenia 158
Low Molecular Weight Heparin 158
Mechanism of Action 158
Therapeutic Range 158
Dosing 158
Reversal and Antidote 158
Side Effects 159
Vitamin K Antagonists 159
Mechanism of Action 159
Monitoring 159
Adverse Effects 159
Reversal and Antidote 160
Antiplatelet Therapy 160
Thrombolytic Therapy 160
Dosing in Thrombolytic Therapy 161
Adverse Effects 162
Treatment of Bleeding 162
Contraindications of Thrombolytic Therapy 162
Treatment of Pediatric Venous
Thromboembolism in Children 162
Treatment of Kawasaki Disease in Children 163
Children Undergoing Fontan Procedure 163
Children with Homozygous Protein C
and S Deficiency 163
22. Stroke in Children 165
Vasant Chinnabhandar, Vinita Jain, SP Yadav,
Anupam Sachdeva
Ischemic Stroke 165
Hemorrhagic Stroke 168
Clinical Features 169
Diagnosis 171
Treatment 172
23. Polycythemia in Newborn 178
Mohammed Ramzan, Anupam Sachdeva,
SP Yadav
Pathophysiology 178
Causes of Neonatal Polycythemia 178
Clinical Features 178
Treatment 179
Complications of Exchange Transfusion 180
24. Blood Component Therapy 181
Vasant Chinnabhandar, VP Choudhary,
Samridh Nagar, Anupam Sachdeva
The Appropriate Use of Blood and
Blood Products 181
Whole Blood 182
Red Blood Cells 182
Special Considerations for Newborns 186
Plasma 187
Special Considerations for Newborns 189
Platelets 189
Granulocytes 192
xxii Practical Pediatric Hematology
Cryoprecipitate 193
Albumin 193
Immunoglobulins 194
Discussion on Special Topics 194
Component and Procedure Specification 195
25. Transfusion Transmitted Infections 199
Deepak Bansal, RK Marwaha
Hepatitis B Virus 199
Hepatitis C Virus 201
Hepatitis D Virus 202
Hepatitis A Virus 202
Hepatitis E Virus 202
Hepatitis GB Virus C/Hepatitis G Virus 202
TT Virus 202
Human Immunodeficiency Virus Types 1
and 2 (HIV1 and HIV2) 202
Human T-Lymphotropic Virus I and II 203
Cytomegalovirus 203
Epstein-Barr Virus 204
Syphilis 204
Malaria 204
Creutzfeldt-Jakob Disease 205
Bacterial Contamination of Blood 205
Rarely Transmitted Parasites 205
Inactivation of Viruses in Blood Products 205
The Indian Scenario 205
26. Supportive Care in Children with Cancer 208
Mohammed Ramzan, Anupam Sachdeva,
SP Yadav
Who Provides Supportive Care? 208
Prevention and Management of Infection in
Immunocompromised Hosts 208
Management 209
Febrile Splenectomized Patients 210
Bacterial Infections 211
Fungal Infections 212
Viral Infections 215
Protozoan Infections 217
Hematopoietic Growth Factors: Basic
Biology of Growth Factors 218
Prevention of Organ Toxicity 221
Pain Management 222
General Guidelines for Management of
Pain in Oncology Patients 224
Management of Nausea and Vomiting 225
Nutritional Support 227
Immunizations 229
Psychosocial Support and End-of-Life Care 230
27. Hematological Manifestations
of Systemic Diseases 232
Himani Manchanda, SP Yadav,
Anupam Sachdeva
Chronic Illness 232
Connective Tissue Disorders 233
Infections 233
Viral Illnesses with Marked
Hematologic Sequel 235
Hematologic Changes Associated with
Specific Infections in the Tropics 238
Hyper-reactive Malarial Splenomegaly
Syndrome 240
Clinical Features 241
28. Disseminated Intravascular Coagulation 247
Mir Sadaqat Hassan Zafar, SP Yadav,
Vinita Jain, Anupam Sachdeva
Epidemiology 247
Etiology 248
Genetic Risk Factors 250
Pathophysiology 250
Clinical Presentation 251
Laboratory Evaluation 251
Diagnosis 253
Differential Diagnosis 254
Scoring System 254
Management (Acute Disseminated
Intravascular Coagulation) 254
29. Disorders of White Blood Cells 259
AP Dubey
Leukocytosis 259
Leukopenia 260
Eosinophilia 260
Eosinopenia 260
Monocytosis 260
Basophilia 260
Lymphocytosis 260
Lymphopenia 261
30. Hematological Changes in HIV Infection 262
Nitin K Shah
Hematological Changes Seen in HIV 262
Pathophysiology 262
Anemia in HIV 262
Leukopenia in HIV 263
Thrombocytopenia in HIV 263
Bone Marrow Changes 263
Coagulation Disorders in HIV 264
Hematological Malignancies in HIV 264
NHL in HIV 264
31. Approach to a Patient with Splenomegaly
and Lymphadenopathy 266
Sunil Narain, LS Arya
32. Myelodysplastic Syndrome in Children 272
Gauri Kapoor, Anshul Gupta
Definition 272
Current Approach to the Classification
of Childhood MDS 272
Primary and Secondary MDS 273
Down’s Syndrome 273
Contributors xxiii
Juvenile Myelomonocytic Leukemia 274
Monosomy 7 274
Fanconi’s Anemia 276
33. Storage Diseases of the
Reticuloendothelial System 281
Nirmalya Roy Moulik, Archana Kumar
Incidence 281
Pathogenesis 281
Genetics 282
Clinical Features 282
Laboratory Diagnosis 282
Morphological Diagnosis 282
Biochemical Diagnosis 286
Genetic Diagnosis 286
Newborn Screening for Lysosomal
Storage Disorders 286
Approach to Diagnosis 286
Principles of Treatment of Lysosomal
Storage Disorders 287
34. Molecular Biology Revolution in Hemato-
Oncology—From Bench to the Bedside 289
IC Verma, Vaishnavi Reddy
Primer of Molecular Biology 289
Tools of the Trade 291
Applications of Molecular Biology 292
35. Hematopoietic Stem Cell Transplantation 298
Mammen Chandy, Biju George, Leni Mathew
Complications 300
Graft Versus Host Disease 300
Alternate Donor Transplantation 302
36. Psychological Support of Chronic
Blood Disorders 305
Roma Kumar, Ushma Rana Singh,
Anupam Sachdeva
Why is Psychological Support so Important? 305
Communication by Doctors and Healthcare
Professionals with Patients 305
Coming to Terms with the Diagnosis 306
Informed Consent 306
Psychological Impact of Admissions
to Hospital 306
Adjustment in the Child with Chronic
Blood Disorders 306
Adjustment in the Family 307
When Treatment Fails 307
Pain Control 307
Self-help Groups 307
37. Vascular Access and Specialized
Techniques of Drug Delivery 309
R Parakh
Indications 309
Types of Devices 309
Purpose of Access 309
Catheter and Port Insertion Techniques 309
Catheter Insertion Complications 309
Catheter Maintenance 310
Technique of Catheter Insertion 310
Problem Solving in Catheter Use 311
Complications 311
Catheter-related Venous Thrombosis 311
Catheter-related Infections 312
Index ......................................................................................................................................................................................... 313