Neonatal Hematology

✍ Scribed by Pedro A. de Alarcón, Eric J. Werner, J. Lawrence Naiman

• Publisher: Cambridge University Press
• Year: 2005
• Tongue: English
• Leaves: 470
• Edition: 1
• Category: Library

✦ Synopsis

Focusing on clinical issues as well as practical approaches to diagnosis and treatment recommendations, this collection represents a practical guide to the pathogenesis, recognition and management of hematologic problems in the neonate. Its contributions are written by practicing physicians specializing in pediatric hematology, neonatology, immunology, pediatric infectious disease and transfusion medicine.

✦ Table of Contents

Cover......Page 1
Half-title......Page 3
Title......Page 5
Copyright......Page 6
Dedication......Page 7
Contents......Page 9
Contributors......Page 11
Foreword......Page 15
Preface......Page 17
1 Neonatal hematology: a historical overview......Page 19
Erythroblastosis fetalis......Page 22
Hemorrhagic disease of the newborn......Page 23
Epilog......Page 24
References......Page 25
Fetomaternal bleeds......Page 28
Twin –twin transfusion syndrome......Page 29
Diabetes......Page 30
Hypertension......Page 31
Maternal malignancy......Page 32
Autoimmune syndromes......Page 33
Maternal medications......Page 34
Iron......Page 35
Folate......Page 36
Cobalamin......Page 37
Toxoplasmosis......Page 38
Syphilis......Page 40
Congenital viral infections......Page 41
References......Page 48
Neonatal erythropoiesis......Page 58
The erythrocyte......Page 60
Hemoglobin......Page 61
Biochemistry of the red cell......Page 63
Postnatal changes in oxygen delivery......Page 64
Initial assessment......Page 65
Blood loss......Page 66
Hemolysis......Page 67
Failure of production......Page 68
Therapy......Page 69
References......Page 71
Minimizing blood loss......Page 76
rhEPO therapy for anemia of prematurity......Page 77
Summary of rhEPO in Prematurity 2004......Page 78
Postnatal iron sources......Page 79
Parenteral iron supplementation......Page 80
References......Page 81
Parvovirus B19......Page 86
Diamond–Blackfan anemia......Page 88
Therapy......Page 89
Pearson syndrome......Page 90
Clinical features......Page 91
Genetics......Page 92
Treatment......Page 93
Dyskeratosis congenita......Page 94
Severe congenital neutropenia......Page 95
Type I......Page 96
Sideroblastic anemia......Page 97
Other rare disorders involving marrow failure in infancy......Page 98
Work-up of a neonate with aplastic anemia......Page 99
References......Page 100
Introduction......Page 109
The Rh antigens......Page 110
Kell......Page 112
MN Ss......Page 113
The maternal antibody response......Page 114
Interactions of antibody with red-blood-cell antigens and the process of red-blood-cell destruction......Page 117
Prevention of maternal isoimmunization......Page 118
The decision to performinvasive monitoring and intervention......Page 121
Delivery of the antibody-sensitized mother and fetus......Page 126
Pathophysiology......Page 128
Diagnosis and management......Page 130
Exchange transfusion......Page 131
Outcome......Page 133
Rh CcEe......Page 134
ABO......Page 135
Duffy......Page 136
References......Page 137
7 Neonatal hemolysis......Page 150
Evidence for anemia and increased red blood cell production......Page 151
Evidence for increased red-blood-cell destruction......Page 152
Infection......Page 153
T-activation......Page 154
Infantile pyknocytosis......Page 155
Hemolysis due to hereditary red-blood-cell membrane disorders......Page 156
Hereditary spherocytosis......Page 157
Hereditary elliptocytosis......Page 159
Glucose-6-phosphate dehydrogenase deficiency......Page 160
Pyruvate kinase deficiency......Page 164
General considerations......Page 165
Alpha thalassemia......Page 166
Beta thalassemia......Page 169
Hemoglobin E/beta thalassemia......Page 170
Sickle cell disease......Page 171
Unstable hemoglobinopathies......Page 172
Methemoglobinemia......Page 173
References......Page 175
Introduction......Page 181
Sickle cell disease......Page 182
Alpha-thalassemia syndromes......Page 184
Carrier of hemoglobin variants......Page 185
References......Page 186
Incidence......Page 189
Definition......Page 191
Plasma proteins......Page 192
Renal blood flow and function......Page 194
Brain blood flow and oxygenation......Page 195
Etiologies......Page 196
Abnormalities of blood volume......Page 197
Renal symptoms......Page 198
Neurologic symptoms......Page 199
References......Page 201
Megakaryocytopoiesis and platelet formation......Page 205
Platelet structure......Page 208
Platelet size......Page 209
Shape changes......Page 210
Aggregation......Page 211
Newborn platelet function assessment......Page 212
Glanzmann thrombasthenia......Page 213
Pseudo-von Willebrand disease......Page 214
oxygenase inhibitors......Page 215
Newborn platelet count......Page 216
Maternal thrombocytopenia......Page 218
Maternal immune thrombocytopenic purpura......Page 220
Neonatal lupus erythematosus......Page 221
Maternal drugs......Page 222
Infant factors......Page 223
Clinical presentation......Page 225
Antigens associated with neonatal alloimmune thrombocytopenia......Page 226
Immune response......Page 228
Therapy......Page 229
Hemolytic disease of the newborn......Page 232
Infection......Page 233
Necrotizing enterocolitis......Page 234
Malformations......Page 235
Fanconi anemia......Page 236
Thrombocytopenia and absent radii syndrome......Page 237
Prognosis......Page 238
Amegakaryocytic thrombocytopenia......Page 241
Wiskott–Aldrich syndrome......Page 242
Metabolic disorders......Page 243
Miscellaneous disorders......Page 244
Diagnostic approach......Page 245
Thrombocytosis......Page 246
REFERENCES......Page 248
Neonatal phagocyte production......Page 272
Normal polymorphonuclear leukocyte function......Page 273
Adhesion......Page 276
Chemotaxis......Page 278
Stress effects......Page 279
Viability......Page 280
Neonatal mononuclear phagocytes......Page 281
Neutropenia......Page 282
Eosinophilia......Page 285
Chronic granulomatous disease......Page 286
Leukocyte adhesion deficiency......Page 287
Glucose-6-phosphate dehydrogenase (G6PD) deficiency......Page 288
Trisomy 21......Page 289
Cytokine treatments......Page 290
References......Page 291
Innate immunity......Page 298
Humoral immunity......Page 302
Cellular immunity......Page 304
Specific immune deficiency disorders presenting in the neonatal period......Page 306
Predominant antibody defects......Page 307
Predominant defects in cell-mediated immunity......Page 309
Combined antibody and cellular immunodeficiencies......Page 310
Diagnosis and management of neonatal immunodeficiencies......Page 312
Diagnostic approach to a neonate with a suspected defect in B-cell function......Page 313
Diagnostic approach to the neonate with a suspected defect in cell-mediated immunity......Page 316
Diagnosis of complement deficiency......Page 320
References......Page 321
Congenital hemorrhagic disorders......Page 328
Acquired hemorrhagic disorders......Page 337
Intracranial hemorrhage......Page 342
Congenital prothrombotic disorders......Page 343
Catheter-related thromboembolic events......Page 347
Non-catheter-related thromboembolic events......Page 348
Anticoagulant and thrombolytic therapy in newborns......Page 349
Acknowledgments......Page 352
References......Page 353
Pretransfusion testing......Page 367
Red blood cell transfusions......Page 368
Anticoagulant-preservative solutions......Page 369
Whole-blood transfusion......Page 371
Donor exposure......Page 372
Recombinant erythropoietin......Page 373
Exchange transfusion......Page 374
Partial exchange transfusion......Page 375
Intrauterine fetal transfusion......Page 376
Platelet transfusions......Page 377
Fresh frozen plasma and cryoprecipitate transfusions......Page 379
Extracorporeal membrane oxygenation......Page 380
Granulocyte transfusion......Page 381
Hemolytic reactions......Page 382
Transfusion-associated graft-versus-host disease......Page 383
Metabolic adverse reactions......Page 384
Transfusion-transmitted infections......Page 385
References......Page 387
Cord-blood procurement and banking......Page 394
Unrelated umbilical-cord blood transplantation......Page 398
References......Page 400
Exposure to maternal malignancy......Page 403
Teratoma......Page 404
Neuroblastoma......Page 405
Leukemia......Page 407
Brain tumors......Page 411
Sarcomas......Page 412
Hepatic tumors......Page 413
Renal tumors......Page 414
Histiocytosis......Page 415
References......Page 416
Red blood cell measurements......Page 424
Morphology......Page 427
Abnormal hemoglobin......Page 428
Iron......Page 429
Platelet morphology and production......Page 431
Platelet-function analysis......Page 432
Coagulation......Page 435
Eosinophils......Page 439
Lymphocytes......Page 441
Progenitor cell assays......Page 442
References......Page 445
Index......Page 449