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Neonatal Hematology
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Details

  • 105 tables
  • Page extent: 468 pages
  • Size: 246 x 189 mm
  • Weight: 1.176 kg

Library of Congress

  • Dewey number: 618.92/01
  • Dewey version: 22
  • LC Classification: RJ269.5 .N46 2004
  • LC Subject headings:
    • Neonatal hematology

Library of Congress Record

Hardback

 (ISBN-13: 9780521780704 | ISBN-10: 0521780705)




Index




A antigens 94

A transferase 94

ABO antigens 94, 97–98, 117–118

   antibody in AB-negative children 98

abruptio placenta 10

acanthocytes 410t

N-acetyl galactosaminyl transferase 94

activated partial thromboplastin time (APTT) 311

activated protein C (APC) 325

acute lymphoblastic leukemia (ALL) 390–391

   in utero origins 390–391

acute megakaryoblastic leukemia (AMkL) 392–393

acute myeloid leukemia (AML) 72, 391

adducin, erythrocyte cell membrane 45t

adenosine deaminase (ADA) deficiency 300, 301

adenovirus, thrombocytopenia 216

adhesion molecules

   ICAM-1 255

   ICAM-2 191, 255

   PCAM-1 191

ADP receptors 191

afibrinogenemia/hypofibrinogenemia 314–315

agglutinating tests, peanut/soya bean 98

agranulocytosis (Kostmann syndrome) 77–78, 265

alkylating agents, hypersensitivity of FA 74

alloantibodies 137

alloimmune hemolysis 135

alpha2-antiplasmin deficiency 318

alpha-fetoprotein 386

   elevated 11, 396

alpha-granules, platelets 191, 197

Alport/Epstein syndrome 224

amegakaryocytic thrombocytopenia (AMT) 77, 223–224

aminolevulinic acid synthase (ALAS-2) 80

amniocentesis 105–107

   algorithm 109t

   antigenic status 103

   and RhD immunization 101

amniotic fluid

   OD (bilirubin) 106t

   RhD typing 103

   spectroscopy 5

anemia

   assessment 47–48

     algorithm 53

   causes

     acquired RBC disorders 132t

     blood loss 48t, 48–49

     bone marrow failure 51t, 50–51

     cord damage 10

     DIC 50

     hemolysis 49t, 49–50

     hereditary RBC disorders 132t

     iatrogenic 49

     physiologic 47, 58, 133

     rubella 25

     toxoplasmosis 21

   congenital dyserythropoietic (CDA) 78–79

   differential diagnosis 47–51

     hemolytic CMV 25

   fetal, diagnosis 11

   fetal hemoglobin, reference ranges 105t

   pernicious 20

   therapy 51

     erythropoietin 51

     transfusion guidelines 351t

     transfusions 350–351

   see also anemia of prematurity; aplastic –; Fanconi –; hypoplastic –; physiologic –; sideroblastic –

anemia of prematurity 47, 58–63

   physiology 58

   therapy 58–60

   vitamin E deficiency 137

animal models, hematopoiesis (mouse embryo) 42t

ankyrin

   erythrocyte cell membrane 45t

   spherocytosis (HS) 140t

anti-A or -B agglutinating substance, IgM antibody 98

antibody

   measurement of titer 98–99

   see also Ig

antibody defects see B cell dysfunction

antibody deficiency

   associated with secondary immune disorders 290–291, 295–298

   management of infants 297–298

   management of mothers 298

   vaccine recommendations 298

antibody-mediated RBC destruction 99–100

anti-cardiolipin antibodies 15

anticoagulant therapy 331–334

   oral anticoagulants 332–333

     protocol 333t

   see also enoxaparin; heparin; reviparin

anticoagulant–preservative (AP) solutions 351–352

   safety 354–355

anticonvulsants

   causing anemia

   thrombocytopenia 205

   vitamin K deficiency 16

anti-D hemolytic disease 49

antigens

   expression by cord blood 94t

   expression in megakaryocytes 189t

   see also A; ABO; B; Duffy; H; HLA; HPA; Kell; Kidd (Jk); MNSs; N; Rh; S

antiglobulin test see direct antiglobulin test (DAT)

antimicrobials, and thrombocytosis 230

anti-phospholipid antibody syndrome 15

antiplasmin, in IDM 13

anti-Rh gamma globulin 5

antithrombin deficiency 326

aortic–gonadal–mesonephric (AGM) region, stem cells 41

aplastic anemia

   drug-induced 70

   Fanconi anemia 73

   hepatitis

   idiopathic 80

   work-up 81t, 81–82

apoptosis

   PMNs 256t

   polymorphonuclear leukocytes (PMNs) 258

Apt test 3

aquaporin, erythrocyte cell membrane 45t

arterial catheters, thromboembolic events 329–330

asphyxia, and thrombocytopenia 206

aspiration pneumonia 226

aspirin 17, 197

   see also NSAIDs

Auto Analyzer 104

autoimmune syndromes 15

B antigens 94

B cell deficiency diseases 289–291

   diagnostic approach 296t, 295–298

B cells

   humoral immunity 284–286

   numbers 284

     assessment 296

   subpopulations, neonates and infants 287t, 288t

Beckwith–Wiedemann syndrome 395, 396

Bernard–Soulier syndrome 195, 220, 224, 416

   platelet glycoproteins (PGPs) 417

beta/gamma switch, HbF 3

beta-methasone, lung maturation 108

BFU see burst-forming unit

Bhutani risk curves, bilirubin 134

bilirubin 110–113

   Bhutani risk curves 134

   OD of amniotic fluid 106t

   pigment assay 106–107, 108

   plasma indirect 113

   and platelet function 198

   unconjugated (indirect) 134

   see also jaundice/hyperbilirubinemia

bilirubin encephalopathy 114

Birbeck granules 397

bleeding time 194, 415–416

blood banks

   cord blood 376–379

     donor confidentiality 378

     private banking 377–378

     public banks 378

blood products, irradiation 272–273, 291, 300

blood sampling

   fetal see cord blood sampling

   minimizing loss 49, 58

     transfusions 351t

blood viscosity 172t, 173t, 174t, 175t

   capillaries 175t

   see also hematocrit

blood volume

   feto-placental circulation 10

   measurement 409

blueberry muffin rash 12, 24, 25, 387, 390

Bombay RBC phenotype 94, 270

bone marrow, stem cell transplantation 376

bone marrow failure 51t, 50–51

   causes 69t

   syndromes 70, 81, 223

bone marrow infiltration, thrombocytopenia 226

Brachmann–de Lange syndrome 81

BRCA1 and BRCA 2 75

breastfeeding

   and jaundice 111

   and UDPGT-1 111

bronchopulmonary dysplasia (BPD) 50, 351

Bruton’s tyrosine kinase (Btk) 289

buffy coat transfusion, neutropenic neonatal sepsis 272

burst-forming unit–erythroid (BFU-E) 40

   yolk-sac 427t

burst-forming unit–megakaryocyte (BFU-Mk) 188t, 188–189

c-mpl see thrombopoietin (TPO) receptor

C-reactive protein (CRP) 282

calcium

   surface membrane depolarization 257

   transient rises in PMNs 257

cancer in newborns 385–397

   brain tumors 393

   epidemiology 385

   hepatic tumors 395–396

   histiocytosis 397

   leukemia 389–393

   neuroblastoma 387–389

   renal tumors 396

   retinoblastoma 395

   teratoma 386–387

   Wilms tumor 396–397

Candida spp.

   C. glabrata sepsis, thrombocytopenia 216

   thrombocytopenia 216

candidiasis, DHST 300

carbon monoxide, generation 134

cardiac defects, TAR syndrome 220

cardiac disease, cord stem cell transplantation 382

cardiopulmonary bypass (CPB), bleeding in 323

cardiopulmonary resuscitation, in HDN 110

cartilage–hair hypoplasia 73, 265

CATCH-22 syndrome, comparison with other syndromes 221t

catheter-related thromboembolic events 329–330

cell-mediated immunity defects 291–292

   diagnostic approach 299t, 298–302

cellular immunity 286–288

central venous lines, thromboembolic events 329

cerebral malformations 225

cervical tumors, Horner’s syndrome 387

CFU see colony-forming unit

Chediak–Higashi syndrome 197, 270–271

chemiluminescence test 104

chemokines 284

   RANTES 284

chemotherapy

   in FA 74

   IUGR 15

   maternal malignancy 14–15

   in sideroblastic anemia 80

   thrombocytopenia 17

chloramphenicol, induction of aplasia 70

chorionic villus sampling (CVS)

   antigenic status 103

   and RhD immunization 101

chromosomal abnormalities, thrombocytopenia 217

chromosome fragility assay see diepoxybutane (DEB)

chronic granulomatous disease (CGD) 268–269

chronic leukemia 391–393

cinchona alkaloids, thrombocytopenia 204

circumcision, bleeding 311, 313, 320

citrate–phosphate–dextrose adenine (CPDA-1) 352, 354

   cord blood 377

Citrobacter diversus 215

clear cell sarcoma of kidney 397

coagulation defects, in cyanotic congenital heart disease 323–324

coagulation proteins see factors

cobalamin deficiency 19–20, 81

   hypokalemia 20–30

   macrocystic anemia 20

   megaloblastic anemia 19

   RDA and stores 20

   thrombocytopenia 226

collagen, platelet activation 193t

collagen defects 416

collagen receptor

   defects 197

   platelet function 191, 192, 193

collagen–epinephrine cartridge assay, platelets 416

colony-forming unit–erythroid (CFU–E) 40

   assays from preterm blood and BM 426t

   yolk-sac progenitors 427t

colony-forming unit–granulocyte erythroid macrophage megakaryocyte (CFU–GEMM) 40

colony-forming unit–granulocyte macrophage (CFU–GM) 255

   assays from preterm blood and BM 426t

   yolk-sac progenitors 427t

colony-forming unit–megakaryocyte (CFU–Mk) 188, 188t, 207

   assays from preterm blood and BM 426t

   cAMT 224

   TAR syndrome 222

colony-stimulating factors see G–CSF; GM–CSF; M–CSF

combined antibody and cellular immunodeficiencies 292–294

combined immunodeficiency disorders

   irradiation of blood products 300

   T cell dysfunction 291–292

common variable immunodeficiency (CVI), in pregnancy 298

complement cascade

   alternative/classical 281

   initiation by IgM 99

complement deficiencies 294

   diagnosis 303t, 302–303

complement system 281–282

   fetus 281–282

   preterm, term, adult 282t

complete blood counts (CBCs) 406

conceptional products, metastatic involvement 385

congenital dyserythropoietic anemia (CDA) 78–79

   treatment 79

   type I 78–79

   type II 79

   type III 79

   type IV 79

Coombs test 49, 53

   direct see direct antiglobulin test (DAT)

   indirect see indirect antiglobulin (Coombs) test (IAT)

copper deficiency 51, 80

cord blood

   antigen expression 94t

   for autologous transfusion 59

   banking 376–379

   and delayed clamping 356

   erythropoietin (EPO) 411, 411t, 412t

   hemoglobin 113

   HLA barriers 376

   thrombopoietin measurement 414

   transplantation see cord stem cell transplantation

   viscosity 172t, 174t, 175t

cord blood sampling 47, 105–107

   absolute lymphocyte count 300

   erythropoietin, and smoking 16

   hemoglobin values 47t

   historical aspects 5

   NAIT 214

   percutaneous umbilical vein sampling (PUBS) 199

   and RhD immunization 101

   for transfusion 349

   ultrasound-guided 107

cord stem cell transplantation 376–382

   advantages of cord blood 376

   banking of cord blood 376–379

     centralized registry 379

     ‘look forward’ programs 379

     public banks 378

     screening 379

   complications

     graft-vs.-host disease (GVHD) 380

     opportunistic infections 380

   donors

     CMV infection 378–379

     availability 376

     consent 377, 378

     private banking 377–378

     types 377

   early experience 380

   inborn errors of metabolism 381–382

   neutrophil engraftment 380

   patient age 381

   unrelated transplantation 380–382

cordocentesis see cord blood sampling

corpus callosum, absence 225

corticosteroids 71, 77

Coumadin 17

cows’ milk intolerance, TAR syndrome 220, 223

Coxsackie virus, thrombocytopenia 216

Creutzfeldt–Jakob disease (CJD), transfusion-related 368

Crigler–Najjar syndrome 111

cryoprecipitate 361–362

   dose 315

   indications 362–363

cutaneous erythropoiesis 12, 24t

cyanosis, methemoglobinemia 157

cyanotic congenital heart disease 323–324

   transfusion 351t

cyclooxygenase inhibitors 197

cytochrome-b5 reductase see NADH-methemoglobin reductase

cytokine therapy 272–273

   cAMT 224

cytokines 283

cytomegalovirus infection 23–25, 69

   in cord stem cell transplantation 378–379

   diagnosis 24

   hematologic manifestations 24

   megakaryocytes 216

   post-transfusion 358, 368

cytopenias, causes 68–81

dacrocytes 410t

dapsone 135

DDAVP

   dose 197

   risk of seizures 198

delayed hypersensitivity skin testing (DHST) 293, 300

dengue virus, thrombocytopenia 216

dermal hematopoiesis 24t

desferoxamine 72

dexamethasone 17

   oligohydramnios and growth retardation 212

di-(2-ethylhexyl)-phthalate (DEHP) 367

diabetes mellitus, maternal 12–13

   see also infant of diabetic mother (IDM)

Diamond–Blackfan anemia 50, 68, 70–72

   diagnosis 71

   mechanism 71

   presentation 71t

   treatment and outcome 71–72

diepoxybutane (DEB) chromosome fragility assay 82

   Fanconi anemia 219

   TAR syndrome 220

DiGeorge syndrome 195, 220, 291

   FISH analysis 300

   treatment 301

2, 3-diphosphoglycerate (2, 3-DPG) 3, 46, 350

   regeneration in PRBC 352

direct antiglobulin test (DAT) 53, 118, 135

   ABO incompatibility 140

directed donor transfusions 355

discocytes 410t

disseminated intravascular coagulation (DIC) 29, 100, 321

   clinical presentation and diagnosis 322

   etiology 321

   hemorrhagic complications 327

   hemostatic complications 361

   management 29, 322

     fresh frozen plasma 227

   mechanism 321–322

   schistocytic anemia 136

   thrombocytopenia 200, 206

   in TMD 392

DNA-based testing, NAIT 211, 212

Dohle bodies 81

Doppler ultrasound 105

Down syndrome (trisomy 21) 81, 271

   leukemia 391

   thrombocytopenia 217

drug-associated disorders

   aplasia 70

   hemolytic anemia 135

   platelet function defects 197–198

   thrombocytopenia 205t, 204–205

drugs, effects on PMNs 263

Dubin–Johnson syndrome 311, 315

Dubowitz syndrome 80

Duffy antigen 95, 99, 118–119

dyskeratosis congenita 76–77, 265

   and thrombocytopenia 219

dyskerin 76

E-selectin, endothelial cells 258

echinocytes 146, 410t

echovirus-11 27

Ehlers–Danlos syndrome 416

electrophoresis, hemoglobin 410

ELISA test, anti-RhD 104

elliptocytes see hereditary elliptocytosis (HE)

elliptocytosis (HE) 138, 138t, 141–142

Embden–Meyerhof pathway 45, 146

embryonal carcinoma 386

endocardial thrombosis syndrome 226

endothelial cells, surface antigen expression 255

enolase 45

enoxaparin

   dosage 332t

   monitoring nomogram 333t

   prophylaxis 332t

enteroviral infections 26–27

   hematologic manifestations 27

enzymopathies 17, 142

eosinophilia 267–268

   associated conditions 268t

   preterm infants 267

eosinophils, adhesion 259

epinephrine receptor, neonatal platelets 192, 195

epsilon-aminocaproic acid (EACA) 218

Epstein–Barr virus infection 30

   thrombocytopenia 216

erythema infectiosum 27, 68

erythroblastosis fetalis

   historical aspects 4–5

   see also hemolytic disease of newborn (HDN)

erythroblasts, nucleated 41

erythrocytes 42–47

   antigen expression in cord blood 94t

   antigens and antibody response 96–99

     see also named antigens

   biochemistry 45–47

   Bombay phenotype 94, 270

   cell membrane 45, 45t

     components 138–139

     glycoprotein 136

   cord 45

   differential count, newborns 410t

   immature 410t

   increased destruction 134–135

   interaction with fibrin 136

   life span 132

   maternal medications 17

   mean cell volume (MCV) 406

   measurements 406–409

   metabolism, summary 143t

   morphology 409–410

   nucleated 133

   osmotic fragility test 139–141

   red cell distribution of width (RDW) 406

   T-activation 136

   transfusions see red blood cell transfusions

erythrocytes, fetal, in maternal blood 11

erythroid progenitors (Ery-CFC) 41

erythroid shapes, differential count in newborns 410t

erythropoiesis 40–42

   cutaneous 12, 24t

   development 41t

   fetal, suppression 359

   growth factors 41t

   and hemoglobin switching 44t

   see also hematopoiesis

erythropoietin (EPO)

   cord blood 411

     preterms 412t

     various gestational ages 411t

   diminished synthesis 350

   and HbF 46

   in IDM 13

   measurement 411

   recombinant 355–356

     early therapy 355

     European Study group trial 356

     US rHuEPO vs. CAP trial 350, 351t

   and smoking 16

   and thrombocytosis 229

   treatment of anemia of prematurity 51

   see also rhEPO

ethanol, and platelet function 198

Ewing’s sarcoma family of tumors (EFT) 394

exchange transfusion 107–108, 113–115, 356–358

   bilirubin values 118

   calculation of blood needed 354t

   complications 357

   donor with sickle trait 154

   fetal 358–359

   followed by thrombocytopenia 214

   G6PD deficiency 145, 146

   in HDN 214, 356–358

   historical aspects 5

   method 358

   neutropenic neonatal sepsis 272

   outcome 115

   partial 357–358

   prenatal 114–115

   survival rates 357

   and thrombocytopenia 206

extracorporeal circulation, Waring blender effect 50

extracorporeal membrane oxygenation (ECMO) 198, 362–363

   blood bank support 349, 363

   TAGVHD in 366

factor V, Leiden mutation 325–326

factor deficiencies 311–318

   V 315

   VII 315–316

   VIII 312–313

     see also von Willebrand factor

   X 316–317

   XI 317

   XII 310

   XIII 317–318

   familial multiple 318

   properties of factors 312t

   replacement therapy in severe hemophilia 314t

   summary 311t

   see also hemorrhagic disorders

factors I to XIII

   available concentrates 313t

   concentration in congenital deficiencies 311t

   concentration in healthy newborns 311t

   properties 312t

Fanconi anemia 50, 73–76

   comparison with other syndromes 221t

   diepoxybutane chromosome fragility assay 82

   genetics 74–75

   international registry 82

   presentation 73t

   thrombocytopenia 218–219

   treatment 75–76

favism 145

Fc receptors 99

   anti-HLA antibody 100

   binding tests 104

   FcgammaRII 100

   IgG-induced RBC destruction 100

   mononuclear phagocytes 263–264

   polymorphonuclear leukocytes (PMNs) 260

Fechtner syndrome 224

ferritin 17

   infants 1–7 months 412t

   measurement 411–413

   in pregnancy 18

   in preterm infant 61

   rhEPO-stimulated eythropoiesis 61, 62

fetal transfusion see exchange transfusion

fetal–maternal alloimmune thrombocytopenia (FMAIT) 208

fetal–maternal hemorrhage (FMH) 10–11, 96

   anti-RhD antigenic sites 100

   causes 11t

   gestational age 96

   and maternal isoimmunization 101–102

feto-maternal transfusion 3, 5

feto-placental unit

   blood volume 10

   hemorrhagic disorders 10–14

fibrinogen

   afibrinogenemia/hypofibrinogenemia 314–315

   concentrate 313t

   deficiency 314–315

   in DIC 322

   structure 314

fibrinogen receptor 192

fibronectins 282

fibrosarcoma, congenital 395

fifth disease 27, 68

filters

   leukodepletion filters 353

   microaggregate filters 353

flow cytometry 82, 102, 104

   platelet function 194–195

   platelet surface receptors 417

   reticulocyte count 133, 410

fluorescence activated cell sorting (FACS) 82, 103

fluorescent treponemal antibodies (FTA-Abs) 23

folate 18–19

   measurement 413

   supplementation 19

folate deficiency 19

folic acid, and erythropoiesis 141

free radicals 46

   Fanconi anemia 74

   iron-induced diseases 63

fresh frozen plasma (FFP) 361

   contraindications 362

   donor retested 361

   dose 314, 315, 362

   indications 361–362

   and NEC 358

   whole blood reconstitution 354t, 353–354

Friedreich’s ataxia 81

fungal infection, thrombocytopenia 216

Fy, a and b antigens 118–119

G-CSF, treatment with 273, 364

galactokinase 45

galactosemia 271

galactosyl transferase 94

gangrene, perinatal 13

GATA 1 locus 225, 392

genotype analysis 82

gestational diabetes 12

giant hemangioma syndrome 217–218

Gilbert syndrome 111–112, 141, 145, 311, 315

Glanzmann thrombasthenia 196t, 195–196, 416, 417

globins 43, 43t, 147–148

   alpha and beta abnormalities 147–148

   alpha globin synthesis 149

   beta globin synthesis 151

glucocorticoids, lung maturation 108

glucose phosphate isomerase (GPI) deficiency 146–147

glucose-6-phosphate dehydrogenase (G6PD)

   function 142–146

   normal/wild-type 142

   racial variants 145

   variants 142

glucose-6-phosphate dehydrogenase (G6PD) deficiency 17, 142–146, 270–271

   causes of hemolysis, drugs 144t

   diagnosis 144–145

   hyperbilirubinemia 145

   mutations

     A-variant 145

     Mediterranean variant 144, 146

   neonatal screening 145

   pyknocytosis 137

   transfusion medicine 146

glucuronyl transferase, reduction 134

glutathione 142

glutathione defective metabolism in phagocytes 271

glutathione peroxidase 45

   deficiency 271

glycogen storage disease IB 271

glycophorin A 95–96

glycoproteins

   erythrocyte cell membrane 136

   see also platelet glycoproteins (PGPs)

GM-CSF

   development of monocytes 258

   treatment with 272

graft-vs.-host disease (GVHD) 72, 75

   cord stem cell transplantation 380

   transfusion-associated TAGVHD 355, 365–366

granulocyte transfusion 272, 363–364

   vs. immunoglobulin replacement therapy 364

granulocytes, irradiation 272

gray baby syndrome 70

gray platelet syndrome 197, 224

growth factors, erythropoiesis 41t

H antigen 94

Haemophilus influenzae infection 216

   splenectomy 141

Hand–Christian–Schuller disease 397

haptoglobin, assay 134–135

   beta-hCG 386

hearing impairment, and thrombocytopenia 225

Heinz bodies 45, 48, 154

   hemolytic anemia 137

HELLP syndrome 14

hemangioepithelioma 395

hemangiomas 50

   eyelid 218

   giant hemangioma syndrome 217–218

   and thrombocytopenia 217

hematocrit

   capillary and venous blood 173t, 409t

   hyperviscosity 172t, 173t, 174t, 175t

   infant of diabetic mother (IDM) 13

   measurement 406

     capillary samples 406

     timing 406, 409

   reduction, partial ET 358

hematocytometers 2

hematopoiesis

   current hypothesis 40

   dermal 24t

   hepatic 41, 42t, 43t, 393

   and megakaryocytopoiesis 187–190

   mesoblastic 43t

   model (mouse embryo) 42t

   myeloid 43t

   phases 40, 43t

   yolk-sac 42, 42t, 254–255

   see also erythropoiesis

hemocytoblasts 40

hemoglobin 43–44

   abnormal 410

   alkali denaturation test 3

   carriers of variants 167–168

   cord blood 113

     preterms 412t

   fetal anemia, reference ranges 105t

   free plasma 134

   globin chain development 43t

   HPLC 153

   isoelectric focusing (IEF) 164, 411

   measurement 406

     heelstick vs. venous 406

     mean cell hemoglobin concentration (MCHC) 406

   oxygen equilibrium curves 46t

   separation by electrophoresis 410

   and smoking 16

   switching 44t

   synthesis, fetal and infant 148t

   unidentified variants 168

     screening 168t

hemoglobin A (HbA)

   oxygen equilibrium curves 46t

   transition from HbF 46, 46t

hemoglobin Barts 147, 149, 150–151

   and MCV 406

   screening 166–167

hemoglobin Constant Spring 148, 150, 151

   genotypes 149t, 151

hemoglobin E/beta thalassemia 152–153, 166

hemoglobin F (HbF) 3

   beta/gamma switch 3

   characteristics 147

   concentration in newborn 133

   and erythrocyte life span 132

   hereditary persistence 11

   normal cord values 47t

   normal values 47

   oxygen affinity 46

   oxygen equilibrium curves 46t

   ‘physiologic nadir’ 47

   reference ranges, anemia and normal 105t

   screening 166t, 164–166, 167t

   synthesis 42, 148t

     iron requirement 61

   transition to HbA 46, 46t

   types observed in newborn screening 153t

hemoglobin F-Poole 155

hemoglobin FM-Fort Ripley 156

hemoglobin FM-Osaka 156

hemoglobin Gower-1 and -2 43

hemoglobin H disease 149–150

   detection 151

hemoglobin Hasharon 155

hemoglobin M disorders 156

hemoglobin Portland 43, 150

‘hemoglobin switching’ 43

hemoglobin–haptoglobin complexes 134–135

hemoglobinopathies 147–155

   carriers 167t

   historical aspects 3

   non-sickle 166

   screening 166t, 163–168

   unstable 154–155

   see also sickle cell syndromes; thalassemia syndromes

hemolysis

   alloimmune 135

     see also hemolytic disease of newborn (HDN)

   severe immune-mediated, and transfusion 365

hemolytic anemia 132

   acquired 135–138

   causes

     acquired RBC disorders 132t

     hereditary RBC disorders 132t

   drug-associated 135

   increased RBC production

   laboratory features 133–135

   in SCD 165

hemolytic disease of newborn (HDN) 91–119

   amniotic fluid OD 106t

   antibodies 92t

hemolytic disease of newborn (HDN)

   antigen-specific considerations 116–119

     ABO 117–118

     Duffy 118–119

     Kell 117

     Kidd (Jk) 119

     MNSs 119

     RhCcEe 116–117

     RhD 116

     third world 49

   causes 49t

   fetal hemolysis by maternal antibody 96–100

     interactions of antibody with RBC antigens 99–100

     maternal antibody response 96–99

     maternal exposure to fetal RBC antigens 96

     transport of antibody across placenta 99

   hemoglobin values, reference ranges 105t

   incidence 91

   management, first affected fetus, algorithm 109t

   maternal risk factors 97t

   paternal testing 103, 109t

   postnatal intervention 108–115

     at delivery 108–109

     management of newborn 110

     partial/whole/later exchange transfusion 114t

   see also exchange transfusion; jaundice/hyperbilirubinemia

   prenatal intervention 100–108

     exchange transfusion 113–115

     invasive monitoring 103–108

     prevention of isoimmunization 100–102

   prenatal management algorithm 109t

   and thrombocytopenia 214–215

hemolytic transfusion reactions 364–365

hemolytic uremic syndrome 29, 50, 136

   atypical 136

   in pregnancy 202

hemophilia

   differentiation from hemorrhagic disease of newborn 6

   factor deficiencies, replacement therapy 314t

hemorrhagic disease of newborn

   differentiation from hemophilia 6

   historical aspects 5–6

hemorrhagic disorders 310–325

   acquired 319–324

   clinical presentation

   congenital 310–318

   in cyanotic congenital heart disease 323–324

   diagnosis 311

   intracranial hemorrhage (ICH) 324–325

   treatment 311

hemosiderin, urinary 135

hemostasis, maternal medications 16

hemostatic abnormalities 310–334

hemostatic proteins 310

heparin

   in ECMO 363

   large vessel thrombosis 322

   LMW, prophylactic 327, 328, 331

   unfractionated (UFH) 322, 331

     administration protocol 332t

heparin-induced thrombocytopenia (HIT) 17, 205

hepatic failure, platelet function 198

hepatic hematopoiesis 41, 42t, 393

hepatic tumors 395–396

hepatitis

   aplastic anemia 70

   bleeding in 323

hepatitis A–C

   transfusion risk 367–368

     estimates 367t

hepatoblastoma 396

hereditary elliptocytosis (HE) 138, 138t, 141–142

hereditary pyropoikilocytosis (HPP) 138t, 141

hereditary RBC disorders 132t, 138–142

hereditary spherocytosis (HS) 138, 138t, 140t, 139–141

Hermansky–Pudlak syndrome 197

herpes simplex infection 26

   delay in cellular immunity 288

   memory T cells 287

   suppression of CFU-Mk 216

hexokinase (HK) deficiency 147

hexosemonophosphate (HMP) shunt 142

histiocytosis 397

HIV infection 29–30, 70, 292

   diagnosis and management 302

   hematologic manifestations

   HIV-1 p24 antigen testing

   thrombocytopenia 202, 216

   transfusion-related 367

     risk estimates 367t

     TAGVHD 366

HLA antigens

   DR

     paternal 100

     super-antigen DRw52 210

   and engraftment 365

   and NAIT 210

   see also major histocompatibility complex (MHC)

Holt–Oram syndrome 220

   comparison with other syndromes 221t

homocysteine

   and cobalamin 19

   in folate deficiency 19

   in IDM 13

homovanillic acid (HVA) 387

Horner’s syndrome 387

Howell–Jolly bodies 48

Hox A11 gene, thrombocytopenia and radio-ulnar synostosis 223

Hoyeraal–Hreidarsson variant, dyskeratosis congenita 76

HPA, classification 209, 209t

HPA-1a antigen 208, 210

   anti-HPA-1a antibody 211

   feto-maternal incompatibility 359

   and risk 212

HPA-1b antigen 208, 209

   and risk 212

HPA-2a antigen 207

HPA-4b (Yuka) 211

HPA-5b antigen 359

HTLV I and II, transfusion risk 367, 367t

human parvovirus B19 27–28

human platelet antigens see HPA

humoral immunity 284–286

hydralazine, thrombocytopenia 204–205

hydrops fetalis

   with anemia 47

   causes 68

   and erythroblastosis fetalis 4

   homozygous alpha thalassemia 150–151

   thalassemias 3

   TMD 391

   treatment 51, 110

   ultrasound 105

hyper-IgM syndrome (HIM) 265, 290

   immunoglobulin replacement therapy 298

hypereosinophilia, with SCID 294

hyperglycemia

   fetal 12

   maternal 12

hyperkalemia, following PRBC 366

hyperkalemic arrest, transfusion-related 366

hypertension in pregnancy 13–14, 204

   see also infant of hypertensive mother (IHM); preeclampsia

hyperthyroidism, and thrombocytopenia 226

hyperviscosity, cord blood 172t, 173t, 174t, 175t

hypocalcemia

   DiGeorge syndrome 195, 220, 291, 300

   transfusion-related 366

hypogammaglobulinemia of infancy (THI) 290, 296–297, 298

   in trisomies 291

hypogammaglobulinemia of prematurity 297

hypoglycemia

   rebound 366

   transfusion-related 366

hypokalemia, cobalamin deficiency 20

hypoplastic anemia 68–82

hypothermia 226

hypovolemia, in TTS 11

ICAMs see adhesion molecules

idiopathic thrombocytopenia (ITP), maternal 202–203, 359

Ig, intravenous Ig 108, 115

   antibody deficiency 297–298

   blood–brain barrier 298

   in NAIT 211–212, 214

   prevention of infections 297–298

   replacement therapy in SCID 301

IgA 286

   maternal deficiency 298

   normal values, term infants to 3 years 285t

   profiles in immunodeficiency disorders 295t

   values

     preterm infants: 25–28 w gestation 283; 29–32 w gestation 284

IgE, profiles in immunodeficiency disorders 295t

IgG 284–285

   anti-D, prevention of maternal isoimmunization 100

   deficiency

     age of documentation 289

     ill preterm infants 285

     and maternal antibody deficiency 284

   maternal antibody half-life in fetus 285

   NAIT antibody, and thrombocytopenia 207

   normal values, term infants to 3 years 285t

   platelet-associated (PAIgG), and thrombocytopenia 206, 215

   profiles in immunodeficiency disorders 295t

   subclasses 286

   values

     preterm infants: 25–28 w gestation 283; 29–32 w gestation 284

IgG anti-RBC antibody 98

   IgG-induced RBC destruction and Fc receptors 100

   production 96

   subclasses 99

IgM 285–286

   anti-A or -B agglutinating substance 98

   anti-T antibodies 136

   antibody in AB-negative children 98

   complement cascade 99

   hyper-IgM syndrome (HIM) 265, 290, 298

   normal values, term infants to 3 years 285t

   production 97

   profiles in immunodeficiency disorders 295t

   uninvolved in HDN 99

   values

     preterm infants: 25–28 w gestation 283; 29–32 w gestation 284

immune globulin see immunoglobulin replacement therapy

immune hemolytic anemia, maternal risk factors 135

immune reconstitution, median absolute values 381t

immune system, immaturity 281t, 280–288

immune thrombocytopenic purpura (ITP), maternal 202–203, 359

immunity

   cellular 286–288

   humoral 284–286

   innate 280–284

   immunodeficiency, centromeric heterochromatin and facial abnormalities (ICF) syndrome 290

   cytogenetics 299

immunodeficiency disorders 280–303

   B cell dysfunction 289–291, 295–298

   classification 289t

   diagnosis and management 294–303

   management

   single gene defects 297t

   T cell dysfunction 291–292

   vaccine recommendations 301

   see also specific named disorders

immunoglobulin replacement therapy

   vs. granulocyte transfusion 364

   hyper-IgM syndrome (HIM) 298

immunoglobulins see Ig

inborn errors of metabolism, cord stem cell transplantation 381–382

indirect antiglobulin (Coombs) test (IAT) 98, 103, 104

   Marsh score 104

indomethacin

   effect on PMNs 263

   prevention of IVH 325

indomethicin 197

   see also NSAIDs

infant of diabetic mother (IDM) 12–13

   polycythemia 12

   thrombocytopenia 13, 225

   thrombosis 55

infant of hypertensive mother (IHM) 14

   neutropenia 14, 265

   polycythemia 14

   thrombocytopenia 14

infant of hypertensive mother (IHM) see hypertension in pregnancy

infantile poikilocytosis 141–142

infections

   congenital 68–70, 135–136

   intrauterine 20–30

   thrombocytopenia 215–216

   transfusion-related 367–369

innate immunity 280–284

integrins

   deficiency, in leukocytes 269

   platelets 191, 193

   PMNs, upregulation 255

interferon alpha-2a, therapy 218

interferon gamma

   activation in mononuclear phagocytes 264

   effect on PMNs 263

   and infection 288

interferons, production 283

interleukins

   IL-1, and thrombocytopenia 217

   IL-3 72

   IL-6 223

   production 283

intracranial hemorrhage (ICH) 324–325

   clinical presentation 324

   in ECMO

   full term newborns 324

   platelet disorders 359

   platelet transfusion-related 359

   preterm newborns 324–325

   thrombolytic therapy-related 334

intracranial see intraventricular

intraperitoneal transfusion, historical aspects 5

intrauterine growth retardation (IUGR)

   and chemotherapy 15

   OD of amniotic fluid 106t

intrauterine infections 20

intrauterine transfusion see exchange transfusion

intravascular hemolysis, anemia 118

intravascular transfusion 358

   vs. intravascular exchange transfusion 358

intraventricular hemorrhage (IVH)

   enteroviral infections 27

   preterm newborns 324

   smoking 16

   and thrombocytopenia 204, 206, 211

     prevention 212

     previous sibling 213

     prognosis 214

intrinsic factor 19

iron

   fetal 18

   perinatal acquisition 61

   postnatal sources 61

   RDA during pregnancy 18

iron deficiency 17–18

   LBW infants 18

   measurement 411–413

iron overload 72

iron saturation ratio 18

iron status of preterm infants 50, 61–63

   cut-off value 61, 62

   long-term status 63

   markers 61

   measurement 61

iron supplementation

   enteral 62

   in HIV 29

   oral high-dose 62

   parenteral 62–63

     toxicity 63

   in rhEPO therapy 60

isoelectric focusing (IEF) 164, 411

isohemagglutinin titer, to ABO antigens 296, 349

Jacobsen syndrome 217

jaundice/hyperbilirubinemia

   approach 113t

   defined 111

   HDN 110–113

     ABO HDN 118

     diagnosis and management 112–113

     Gilbert syndrome 111–112

   in HS 139

   management 141

   pathophysiology 110–113

   physiologic 134

   PK deficiency 146

   prevention in G6PD 145

   see also hemolytic disease of newborn (HDN)

Jk antigen 95, 119

juvenile myelomonocytic leukemia 391

kaposiform hemangioendothelioma 217

Kasabach Merritt syndrome (KMS) 217–218

Kell antigen system 92, 94–95, 99, 117

keratocytes 410t

kernicterus 112–113

Kidd (Jk) antigen 95, 99, 119

Klebsiella infection 215, 363

Kleihauer–Betke technique 11

   modification for HbF 102

knizocytes 410t

Kostmann syndrome (agranulocytosis) 77–78, 265

L-selectin

   mononuclear phagocytes 259t, 263

   PMNs 255, 258, 259

lactic dehydrogenase (LDH), marker of accelerated RBC destruction 134

lactoferrin 282–283

Langerhans cell histiocytosis 397

large vessel thrombosis, heparin 322

LBW infants, iron deficiency 18

Letterer–Siwe disease 397

leukemia 389–393

   ALL 390–391

   AML 72, 391

   chronic 391–393

   clinical presentation 390

   diagnosis 390

   Down syndrome (trisomy 21) 391

   juvenile myelomonocytic leukemia 391

   maternal, and cancer in newborns 386

   transient myeloproliferative disorder 390, 391–393

leukemia cutis 390

leukemoid reactions 390

   TAR syndrome 220

leukocyte adhesion deficiency (LAD-1 and -2) 269–270

leukocytes

   and maternal medications 17

   transfusions 272

leukocytosis, TAR syndrome 220

leukodepletion filters 353, 368

leukodystrophies, cord stem cell transplantation 382

Li–Fraumeni syndrome 395

Liley curve 106–107

lipid emulsions, effect on PMNs 263

lipopolysaccharide binding proteins 283

LISS antiglobulin technique 118

Listeria infection 215, 283

liver disease, bleeding in 323

lumiaggregometry 416

lung injury, transfusion-associated (TRALI) 365

lupus anticoagulant 15

lupus erythematosus

   neonatal 203

   neonatal syndrome (NLE) 203

lymphocytes

   [K] ADCC assay 104

   subpopulations, neonates and infants 287t, 288t

   see also B cells; T cells

lysosomal assembly defects 197

lysosomal storage disorders 271

M-CSF, development of monocytes 258

Mac-1 deficiency (leukocyte integrin deficiency, LAD-1) 269–270

macrocystic anemia, cobalamin deficiency 20

macrothrombocytopenias 224

major histocompatibility complex (MHC), class II deficiency 291–292

malaria, transfusion-related 368

Malassezia fungemia, thrombocytopenia 216

malformations, and chemotherapy 15

malignancy, maternal 17

malignant melanoma, metastasis 15

mannose binding proteins 283

   alpha-mannosidosis 271

Marfan syndrome 416

Marsh score 104

maternal antibodies, HDN 92t

maternal isoimmunization, prenatal interventions in HDN 100–102

maternal malignancy 14–15

   and cancer in newborns 385–386

maternal medications 16–17

maternal RBC transfusion history 96

maternal risk factors 12–30

   drug-associated 135

   HDN-associated 97t

   immune hemolytic anemia 135

May–Hegglin anomaly 224

mean corpuscular volume (MCV) 133

   screening 168

mean platelet volume (MPV)

   in infection 215

   normal 191

   normal newborn 192

meconium aspiration syndrome 226

Mediterranean macrothrombocytopenia 224

megakaryocytes

   antigen expression 189t

   classification 189

   colonies in newborn 189–190

   measurement 413–414

   polyploidization and maturation 190t

   proplatelets 189t, 190

   see also megakaryocytopoiesis; platelet(s)

megakaryocytopoiesis 188t, 187–190

   differention markers 189

   platelet formation 190

megaloblastic anemia, cobalamin deficiency 19

2-mercaptoethanol 98

mesoblastic nephroma 396

metabolic disorders, thrombocytopenia 225–226

metaphyseal dysostosis 76

metastatic involvement, conceptional products 385

methemoglobinemia 45, 155–157

   acquired 155–156

   congenital 156

   cyanosis 157

   renal tubular disease 156

   treatment 157

methylene blue 157

methylmalonic acid, folate deficiency 413

mevalonic acidemia 226

microangiopathic (schistocytic) anemias 136

middle cerebral artery (MCA), velocity studies 105, 107

mitochondrial DNA, Pearson syndrome 72

Mk-CSA 230

MLL gene 390

MN blood group system 95–96

MNSs antigen, HDN 119

monocyte monolayer adherence assay (MMA) 104

monocytes see mononuclear phagocytes

mononuclear phagocytes

   abnormal/dysfunction 268–271

     management

   activation by INF-gamma 264

   adults 258

   C3b receptor expression 263

   candidacidal ability 264

   Fc receptor expression 263–264

   L-selectin expression 263

   microbicidal ability 264

   myeloperoxidase deficiency 270

   neonates 258–263

   as precursor cell 258

   similarity to PMNs 258

monosomy-7 syndrome 391

Montreal platelet syndrome 224

mucopolysaccharidoses, cord stem cell transplantation 382

myelodysplasia 391

myeloid growth factors, PMN storage pools at delivery 254

myeloperoxidase

   deficiency 270

   in PMNs 260

myelopoiesis

   at delivery 254

   placental inhibitor 265

N antigen, HDN 119

NADH-methemoglobin reductase 155

   deficiency 156–157

NADPH oxidase

   activation by PMNs 256t

   deficient/absent 268

   PMNs 257

necrotizing enterocolitis (NEC) 136, 137

   and FFP 358

   and thrombocytopenia 206, 216–217, 227

neonatal alloimmune thrombocytopenia (NAIT) 201, 207–214

   antigens associated 208–209, 209t

   assessment of risk 212

   clinical presentation 207–208, 208t

   complications 214

   diagnostic approach 227–228, 229t

   differential diagnosis 211

   DNA-based testing 211

   genetic diagnosis of infant at risk 212

   immune response 210–211

   incidence and prevalence 209–210

   mechanism 206t

   in mothers 202

   platelet count 207

   therapy 211–212

     platelet transfusions 360t, 359–361

     prenatal 212–214

     tailored to risk categories 213, 214t

neonatal autoimmune thrombocytopenia (NITP) 361

neonatal lupus see lupus erythematosus (NLE)

neuraminidase 136

neuroblastoma 387–389

   diagnosis 387–388

   presentation 387

   screening 389

   staging 388, 388t

   treatment 389

neutropenia 264–267

   acquired forms 265

   algorithm for treatment 259t

   associated conditions 265t

   causes in neonates 259t

   defined 363

   delayed separation of umbilical stump 270

   infant of hypertensive mother (IHM) 14, 265

   and infection 265–267

   inherited congenital 265

neutropenic neonatal sepsis 272

neutrophilia 267

   associated conditions 267t

neutrophil

   counts

     preterm infants, 0–60 hours, and 2.5–28 days 423t

     term and preterm infants: 0–60 h 419; 60–120 h, and 5–28 days 420

   engraftment, cord stem cell transplantation 380

   storage pool 363

   see also mononuclear phagocytes; polymorphonuclear leukocytes (PMNs)

nitric oxide 198

nitrites 155

nitroblue–tetrazolium (NBT) test 268, 269t

Noonan syndrome 81

NSAIDs 204

nutritional deficiencies, maternal 17–20

oligohydramnios, in TTS 12

oligohydramnios and growth retardation, dexamethasone 212

Omenn syndrome 294

   flow cytometry 300

ophthalmoplegia, and thrombocytopenia 225

opsonins

   PMNs 261

   preterm infants 281

   production 281

osteopetrosis 78

oxygen delivery, postnatal changes 46–47

P antigen 27

P-selectin 191

   endothelial cells 258

packed red blood cells (PRBC)

   in ECMO 363

   formulae for transfusion 354t

   guidelines 351t

   hyperkalemia 366

   infusion issues 353

   optimal volume

   partial ET 357–358

   shelf life 352

   whole blood reconstitution 353–354

   see also red blood cell transfusions

pancytopenia

   congenital 218

   Fanconi anemia 73

   workup 81–82

Pappenheimer bodies 48

Paris–Trousseau syndrome 224

parvovirus B19 27–28, 68–69

   fetal and perinatal transfusions 69

   thrombocytopenia 216

paternal HLA DR antigens 100

paternal RBC transfusions 355

paternal testing, HDN 103

PCAMs see adhesion molecules

Pearson syndrome 72–73

pentose phosphate pathway 45

pentoxifylline, effect on PMNs 263

percutaneous umbilical vein blood sampling see cord blood sampling

peripheral blood smear 133

   elliptocytosis 141

   examination procedure 48

   pyknocytosis 137–138

pernicious anemia 20

PFA-100\myregistar, measurement of hemostasis 194

phagocytes see mononuclear; polymorphonuclear leukocytes

phagocytosis

   frustrated 257

   in PMNs 261

phenylketonuria, screening 163

phorbol myristate acetate (PMA), NBT reduction 268

phosphatidylglycerol, amniotic fluid 108

phosphatidylserine (PS) 195

phosphofructokinase 45

phosphofructokinase (PFK) deficiency 147

phosphoglycerate kinase (PGK) 45

   deficiency 147

phospholipase C (PLC) 193

phototherapy

   bilirubin levels 114, 115, 118

   G6PD deficiency 145

   platelet function 198

physiologic anemia 47, 133

   hemoglobin F (HbF) 47, 58

   transfusions 350

physiologic hyperbilirubinemia 134

placenta, immune function 283

placenta previa 10

placental abnormalities, thrombocytopenia 205

placental blood

   autologous transfusion 356

   heparinized 356

placental infarction 200

plasma volume, measurement 409

plasmapheresis 108

Plasmodium vivax 95

plasticizers, di-(2-ethylhexyl)-phthalate (DEHP) 367

platelet activating factor (PAF), in NEC 217

platelet activation, by collagen 193t

platelet adhesion 416

platelet anatomy 191

   normal newborn 192

platelet count 198, 413–414

   fetus 199, 200t

   full term 198, 198t, 199t

   infant factors 205–215

   maternal factors 200–203

     diagnosis and incidence 201t

     drug-associated thrombocytopenia 204–205

     drugs 204–205

     incidence (fetal/neonatal) 202t

   platelet transfusions 227–228, 359–361

     intrauterine 214, 359

   preterm infant 198, 199t

   thrombocytosis 228t, 228–229, 230

platelet formation 190t

   production 413–414

platelet function 192, 414–417

   adhesion 193

   aggregation 193, 194

   assessment 194

   bleeding time 194, 415–416

   flow cytometry 194

   normal newborn 194–195

   reactivity in IDM 13

   shape changes 192

platelet function defects 195–198, 228

   collagen receptor defects 197

   congenital platelet activation defects 197

   drug-induced 197–198

   and intracranial hemorrhage (ICH) 359

   other conditions 198

   presenting in older children 196–198

   secretory disorders 197

   therapy 198

platelet glycoproteins (PGPs) 189–190, 191, 192

   Bernard–Soulier syndrome 417

   binding 193

   binding by collagen 193t

   classification 209t

   Glanzmann thrombasthenia 195, 417

   HPA antigens 209

   vWFR defect 195

platelet release 416

platelet size 191–192

   giant 413

   normal newborn 192

platelet structure 190–192, 413–414

   alpha-granules 191, 197

   normal newborn 192

platelet surface 191

   normal newborn 192

platelet transfusions 198, 227–228, 359–361

   choice of product 360

   congenital platelet activation defects 197

   Glanzmann thrombasthenia 196

   guidelines 360t

   indications 227–228

   intrauterine 214, 359

   maternal platelets 360

   and NAIT 211–212, 213–214, 360t, 359–361

   neonatal alloimmune thrombocytopenia 359–361

   prophylactic 359

   random donor 361

platelet unit, standard 360

platelet pheresis 360

Pneumocystis carinii pneumonia (PCP, prophylaxis 298

poikilocytosis 142

polychromatophilic cells 48

polycythemia

   historical aspects 2

   infant of diabetic mother (IDM) 12, 225

   infant of hypertensive mother (IHM) 14

   and smoking 16

   and thrombocytopenia 226

polycythemia–hyperviscosity syndrome 11–12

   complications 12, 13

   partial ET 358

polyhydramnios

   in HDN, indication for delivery 109

   in TTS 12

polymorphonuclear leukocytes (PMNs) 254–273

   activation 257

   adhesion 258–260

   anti-viral and cytotoxic activity 263

   apoptosis 258

   chemoattractants 257

     receptors 257

   chemotaxis 260–261

   drug effects 263

   dysfunction

     management 272–273

     sites of abnormality 259t

   exudation 257

   Fc receptor function 260

   half-life 255

   L-selectin 255, 258, 259

   myeloperoxidase content 260

   normal function

     adults 255–258

     components 256t

     neonates 258–263

     phagocytosis and bacterial killing 261

   numbers 264

     VLBW infants 264

   opsonins 261

   primary granule markers 260

   primary granules 260

   production 254–255

   reactive maternal antibodies 265

   respiratory burst activity 262

   rolling behaviour 255–257, 258

   signal transduction 261

   storage pool 254–255

     circulating/marginated 255

   stress effects 261–262

   structure

     cytoskeleton 261

     secondary granules 255, 257, 260

     tertiary granules 260

   surface antigen heterogeneity 260

polymorphonuclear leukocytes (PMNs)

   surface receptor expression 260

   viability 262

portal vein thrombosis 329

pre-eclampsia/eclampsia

   HELLP syndrome 14

   thrombocytopenia in newborn 204

   TPO in nonthrombocytopenic term and preterm infants 414t

pregnancy termination, anti-D 101

preservatives, anticoagulant–preservative (AP) solutions 351–352

primitive neuroectodermal tumor (PNET) 394

prion disease, transfusion-related 368

propionic acidemia 226

proplatelets 189t, 190

   formation 190t

prostacyclin, in IDM 13

protein C

   activated (APC) 325

   deficiency 327–328

   and DIC 322

protein S deficiency 327, 328

prothrombin

   deficiency 6, 310, 315

   gene G20210A 326

prothrombin complex concentrates (PCC) 315

prothrombin time 311

prothrombotic disorders 325–328

protoporphyrin 18

   infants 1–7 months 412t

protoporphyrin/heme ratio 61, 62

pseudo von Willebrand disease 196–197

pulmonary disease, transfusion 351t

pulmonary hypertension 226

pure white cell aplasia 265

purine salvage pathway dysfunction in SCID 293–294

purpura fulminans 327–328

pyknocytosis 137–138

pyrimidine 5’nucleotidase (P5’N) deficiency 147

pyropoikilocytosis (HPP) 138t, 141

pyroxidine deficiency 80

pyruvate kinase deficiency 146–147

   pyknocytosis 137

radial dysplasia, TAR syndrome 219

radial hypoplasia, and thrombocytopenia 225

radiation therapy

   FA 74

   in pregnancy 15

RANTES 284

red blood cell transfusions 59, 350–351

   2, 3-DPG in 352

   formulae for transfusion 354t

   paternal 355

   PRBC infusion issues 353

   PRBC shelf life 352, 354–355

   whole blood reconstitution 354t, 353–354

   see also packed RBCs

red blood cells see erythrocytes

red cell distribution width (RDW) 2

renal failure, platelet function 198

renal tubular disease 156

renal tumors 396

   clear cell sarcoma 397

   mesoblastic nephroma 396

   rhabdoid tumor 397

   WAGR 396

   Wilms tumor 396–397

renal vein thrombosis 226, 330

   IDM 13

respiratory burst activity, PMNs 262

respiratory distress syndrome 108, 110

reticular dysgenesis 77

reticulocytes

   cord blood percentage, preterms 412t

   count 133, 410

     and cord clamping 410

retinal hemorrhage 327

retinoblastoma 395

reviparin

   dosage 332t

   monitoring nomogram 333t

   prophylaxis 332t

Rh antibodies, formation 5

Rh antigens 92–94

   C/c antigens 93–94, 116–117

   E/e antigens 93, 116–117

   Rh-related antigen LW 94

   RhD gene and antigen 93t, 92–94

Rh factor 4–5

   RhCE and RhD 93t

rhabdoid tumor of kidney 397

rhabdomyosarcoma 394

RhAG, Rh-associated glycoprotein 93

RhCE gene and antigen 92, 93t

RhD

   algorithm, approach to management 109t

   anti-RhD

     dose (by country/timing) 102t

     dose and response 100

     half-life 101

     and HDN 116

     IAT titer 104

     postnatal injection 100, 101

     pre- and postnatal clinical trials 101

     prophylaxis against sensitization 97, 101

     standard dose 102

   epitopes 92–93

   frequency 92

     decrease in immunization rate of at-risk mothers 101

   ‘grandmother theory’ 102

   and HDN 91–92

   in vitro fertilization 102

   nonresponders 97

   see also hemolytic disease of newborn (HDN)

rhEPO therapy

   for anemia of prematurity 59–60

   cost 60

   with parenteral iron 62

   side effects 60

   trials 60

Roberts syndrome 220

   comparison with other syndromes 221t

rubella 25–26

   hematologic manifestations 25

S antigen, HDN 119

sacrococcygeal teratoma 386–387

sarcomas

schistocytic anemia 136

   DIC 136

schizocytes 410t

sclerosing agents 218

screening

   G6PD deficiency 145

   hemoglobinopathies 163–168

   phenylketonuria 163

   sickle cell syndromes 163, 164

   thalassemia syndromes 166

screening programs 164–168

Sebastian syndrome 224

Seckel syndrome 80

secretory platelet disorders 197

serine proteinase inhibitors 327

severe combined immunodeficiency disorders (SCID) 293

   IV IgG replacement therapy 301

   with hypereosinophilia 294

   Ig and lymphocyte profiles 295t

   purine salvage pathway dysfunction 293–294

   therapy 301

   X-linked SCID 293, 301

   see also ADA; Omenn syndrome; Zap-70 deficiency

severe congenital neutropenia (SCN) 77

   international registry 77

shear rate see blood viscosity

Shwachman–Diamond(–Oski) syndrome 76, 265

sialic acid/sialoglycoproteins 136

sickle cell syndromes 153–154

   hemoglobin S-C disease 153

   infants of mothers with S-C disease 154

   screening 163, 165t, 164–166

   sickle beta\circ thalassemia 153

   sickle cell anemia 153–154

sideroblastic anemia 50, 72, 79–80

signal transduction pathways, X-linked SCID 293

Singer 1-minute alkali denaturation test 3

sinovenous thrombosis 330

Sjogren syndrome 204

small-for-gestational age (SGA) infant, thrombocytopenia 226

smoking 16

   passive 16

Sn-mesoporphyrin 145

solvent detergent (SD) treated plasma 361

spectrin

   erythrocyte cell membrane 45t

   spherocytosis (HS) 140t

spherocytes see hereditary spherocytosis (HS)

spherocytosis (HS) 138, 138t, 140t, 139–141

splenic sequestration crisis 154

splenomegaly, in HS 139

Ss blood group system 95–96

SS-A, anti-Ro 15

SS-B, anti-La 15

Staphylococcus aureus, coagulase negative 216

stem cell transplantation, donor availablity 376

stem cells 40–42

   aortic–gonadal–mesonephric (AGM) region 41

   erythropoiesis 41t

   megakaryocytopoiesis–thrombocytopoiesis 188t

   transplantation 72, 75, 76

   see also cord stem cell transplantation

storage pool defects 197

Streptococcus pneumoniae infection 136

   B beta-hemolytic 215

     T-cell dysfunction 287

   splenectomy 141

streptokinase 334t, 333–334

stress

   labor-induced 261–262

   and PMNs 261–262

sulfonamides, thrombocytopenia 204

superoxide dismutase 46

surfactant 108

syphilis 22–23

   congenital 23

T cells 286–288

   absence of memory T cells 286

   and combined immunodeficiency 291–292

   cytolytic activity decrease 288

   naive phenotype 287

   specific immunodeficiency

     associated conditions 294

     laboratory findings 299

     management 300–302

     thymus size 299

   subpopulations, neonates and infants 287t, 288t

T-activation 136–137, 365

   suspected 136

T-cell receptor diversity 287

TAR syndrome see thrombocytopenia and absent radii (TAR) syndrome

teratoma 386–387

thalassemia syndromes 148

   alpha thalassemia 148–151

     genotypes 149t

     homozygous (hydrops) 150–151

     screening 167t, 166–167

     trait 149

   beta thalassemia 151–152

     homozygous 151–152

   gamma thalassemia 153

   hemoglobin E/beta thalassemia 152–153

   hemoglobin H disease 149–150

   hydrops fetalis 3

   screening 166, 166t

   silent carrier state 149

thiazides, thrombocytopenia 204

Thompsen–Friedenreich antigen 365

thrombasthenia, Glanzmann 196t, 195–196

thrombin

   activation of platelets 194

   and arachidonic acid 195

   arachidonic acid production 195

thrombin receptor 191

thrombocytopenia 198–228

   amegakaryocytic 77, 223–224

   diagnostic approach 227–228, 229t

   drug-associated 205t, 204–205

   etiology

     chemotherapy 17

     in sick newborn 228t

   heparin-induced (HIT) 17, 205

   immune 200

   incidence 207t

   infant of diabetic mother (IDM) 13

   infant of hypertensive mother (IHM) 14

   infections 215–216

   inherited disorders 225t, 218–225, 228

     X-linked 225

     chromosomal abnormalities 217

   isolated perinatal, secondary to maternal ITP 202–203, 359

   malformations 217–218

   maternal thrombocytopenia 200–203

     causes 200

     diagnostic approach in infant 229t

     drug-associated 205t, 204–205

     gestational 201

     platelet count 228

   mechanism 206t

   metabolic disorders 225–226

   neonatal autoimmune (NITP) 361

   pseudothrombocytopenia 413–417

   severe, defined 227

   transient 15

   treatment 227–228

   see also neonatal alloimmune thrombocytopenia (NAIT)

thrombocytopenia and absent radii (TAR) syndrome 50, 77, 80, 219–223

   comparison with other syndromes 221t

   features, compared with other studies 222t

   pathogenesis 222–223

   prognosis 220–222

   storage pool defects 197

   treatment 223

thrombocytopenia and radio-ulnar synostosis 223

thrombocytopoiesis 188t

thrombocytosis 228–230

   essential (ET) 230

thromboembolic disorders 325

   catheter-related 329–330

   etiology 325

   non-catheter-related 330

   prothrombotic disorders 325–328

thrombolytic therapy 331–334

   agents 334t, 333–334

thrombopoietin receptor (c-mpl) 190, 222–223, 224

   mutations 224

thrombopoietin (TPO)

   elevated

     in cord blood 215

     thrombocytosis 228

   measurement 414

   in nonthrombocytopenic term and preterm infants, mothers with pre-eclampsia 414t

thrombosis

   IDM 13

   infant of diabetic mother (IDM) 13

thrombotic thrombocytopenic purpura (TTP) 29, 50

   in pregnancy 202

   purpura fulminans 327–328

thromboxane A2 (TxA2) 194

thymic hypoplasia 299

thymus, immune reconstitution 381

tissue plasminogen activator (tPA) 333, 334t

tolbutamide, thrombocytopenia 205

TORCH syndrome 216

total parenteral nutrition, rhEPO in 60

toxoplasmosis 20–22

TPN see total parenteral nutrition

tranexamic acid 218, 325–334

transcobalamin deficiency 50, 81

transferrin receptor 18, 61

   infants 1–7 months 412t

   measurement 412

transferrin saturation ratio 412

transfusion 349–369

   adverse reactions 364–369

     hemolytic reactions 364–365

     infections 367–369

     leukocyte alloimmunization 365

     lung injury (TRALI) 365

     metabolic 366–367

     TAGVHD 355, 365–366

   autologous 356

   complications 354–355

   current risk estimates 367t

   directed donor transfusions 355

   emergency 110

   multiple, donor exposure 354–355

   pre-transfusion testing 349–350

   see also exchange –; feto-maternal –; maternal –; platelet –; and named products

transient erythroblastopenia of childhood (TEC) 71

transient hypogammaglobulinemia of infancy (THI) 290

   diagnosis 296–297

   evaluation 298

   in trisomies 291

transient myeloproliferative disorder 390, 391–393

   hydrops fetalis 391

   spontaneous regression 393

treponemal antibody tests 23

triose phosphate isomerase (TPI) deficiency 147

trisomies

   hypogammaglobulinemia of infancy (THI) 291

   and thrombocytopenia 217

tumor necrosis factor (TNF)

   and thrombocytopenia 217

   TNF-alpha 283

Turner syndrome 81, 217, 290

twin–twin transfusion syndrome (TTS) 11–12

   incidence 11

   management 12

tyrosine kinase receptors 191

ultrasound, in HDN 105

umbilical arterial catheters, thromboembolic events 329

umbilical cord

   damage 10

   delayed clamping 59, 356, 406, 409

   mummification 270

   see also cord blood; cord stem cells

umbilical stump, excessive hygienic care 270

umbilical venous catheters, thromboembolic events 329

Upshaw–Schulman syndrome 226

uridinediphosphoglucuronate glucuronosyltransferase (UDPGT-1) 110–112, 145

   and breastfeeding 111

   mutations 111–112

urokinase 333, 334t

vaccines

   recommendations

     antibody deficiency 298

     immune deficiency 301

vanillylmandelic acid (VMA) 387

vascular anastomoses 11

velamentous cord insertion 10, 11, 49

venous catheters, thromboembolic events 329

viral infections 23–30

vitamin B12 deficiency 19–20

   measurement 413

vitamin E deficiency 137

   pyknocytosis 137

   supplementation 137

vitamin K

   mechanism of action 319

   prophylactic administration 320–321, 325

     recommendations 321t

   sources 319

vitamin K deficiency 16, 319–321

   clinical presentation 319, 320t

   historical aspects 6

   IVH 325

   treatment 319–320

von Willebrand disease 416

von Willebrand factor receptor (vWFR) 191, 192

   adhesion 193

   defect, Bernard–Soulier syndrome 195

   pseudo vWD 196

von Willebrand factor (vWF)

   deficiency 316

     prenatal diagnosis 311

     vWF–factor VIII replacement therapy 317t

   vWF-mediated binding of collagen 193t

Wilms tumor 396–397

Wiskott–Aldrich syndrome 197, 224–225, 292–293

   complications 301

X-linked agammaglobulinemia (XLA) 265, 289–290

   diagnosis 297

X-linked and other SCID 293, 301

X-linked thrombocytopenias 225

yolk-sac

   hematopoiesis 42, 42t, 254–255

   progenitors vs. age of development 427t

yolk-sac endoderm 41

yolk-sac tumor 386

Zap-70 deficiency 300

zidovudine 29

zinc deficiency 51

zinc protoporphyrin see protoporphyrin


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