Preface viii
Abbreviations x
1 The full blood count and blood film in healthy term and preterm neonates 1
Introduction 1
Brief outline of the ontogeny of haemopoiesis 1
Properties of fetal haemopoietic stem and progenitor cells 2
Red blood cell production and development in the fetus and neonate 5
Erythropoietin production in the fetus and neonate 6
Haemoglobin synthesis and red blood cell production in the fetus and newborn 6
Red blood cell lifespan and the red blood cell membrane in the fetus and neonate 9
Red blood cell metabolism in the fetus and neonate 10
Iron metabolism in the fetus and neonate 11
Normal values for red blood cell parameters in the fetus and neonate 12
Leucocytes in the fetus and newborn 17
Leucocyte production and function in the fetus and neonate 18
Platelets and megakaryocytes in the fetus and neonate 23
Developmental megakaryopoiesis and thrombopoiesis 23
Platelet numbers in the neonate and fetus - normal values 24
Neonatal platelet function 25
Practical problems in interpreting neonatal blood counts and films 25
Sample quality/artefacts 25
Site of sampling 26
Gestational age and postnatal age 27
Pregnancy-associated complications and mode of delivery 28
References 28
2 Red cell disorders: Anaemia, jaundice, polycythaemia and cyanosis 38
Neonatal anaemia 38
Definition and clinical significance of neonatal anaemia 38
Neonatal anaemia due to reduced red cell production 39
Neonatal anaemia due to increased red cell destruction 47
Neonatal anaemia due to haemoglobinopathies and other microcytic anaemias 67
Neonatal anaemia due to blood loss 76
Anaemia of prematurity 81
A simple diagnostic approach to neonatal anaemia 83
Neonatal polycythaemia 85
Haematological causes of cyanosis 86
Principles of red cell transfusion in neonates 86
Illustrative cases 88
Abbreviations used in the illustrative cases 110
References 110
3 Neonatal infection and leucocyte disorders 128
Leucocyte abnormalities in neonatal systemic disease 128
Increases in leucocyte numbers 128
Neonatal infection and its differential diagnosis: clues from the blood count and blood film 135
Storage disorders: diagnostic clues from the blood film 144
Neonatal neutropenia 145
Definition and causes of neutropenia 145
Immune neutropenia 148
Inherited congenital or neonatal neutropenia 150
Haematological features of neonates with Down syndrome 154
Congenital leukaemia 154
Leukaemia and preleukaemia in neonates with Down syndrome 156
Acute leukaemia in neonates without Down syndrome 160
Juvenile myelomonocytic leukaemia and Noonan syndrome myeloproliferative disorder 168
Illustrative cases 169
Abbreviations used in the illustrative cases 176
References 176
4 Disorders of platelets and coagulation, thrombosis and blood transfusion 190
Thrombocytopenia overview 190
Causes of neonatal thrombocytopenia: a practical classification based on age at onset 192
Fetal thrombocytopenia 194
Early-onset neonatal thrombocytopenia (presenting at <72 hours of age) 194
>72 hours of age) 196
Conditions leading to clinically significant thrombocytopenia in the neonate 197
Fetal/neonatal alloimmune thrombocytopenia 197
Neonatal thrombocytopenia due to maternal autoimmune disease 200
Thrombocytopenia due to congenital infections 201
Neonatal thrombocytopenia associated with chromosomal abnormalities 205
Inherited thrombocytopenia 206
Investigation of neonatal thrombocytopenia 215
Management of neonatal thrombocytopenia 215
Indications for platelet transfusion 216
Platelet function disorders 217
Inherited platelet function disorders with normal platelet counts presenting in the neonate 218
Thrombocytosis 218
Abnormalities of coagulation 220
Developmental haemostasis 220
Laboratory investigation of coagulation disorders in the neonate 220
Acquired coagulation abnormalities 221
Inherited coagulation disorders 223
Thrombosis 224
Screening tests for thrombophilia in neonates 224
Acquired thrombotic abnormalities 225
Inherited thrombotic abnormalities 225
General principles of neonatal platelet and plasma component transfusion 226
Platelet transfusion 226
Fresh frozen plasma 226
Cryoprecipitate 227
Prothrombin complex and recombinant factor VIIa 227
Illustrative cases 227
Abbreviations used in the illustrative cases 233
References 234
Index 254
