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Index

Note: Page references in italics refer to Figures; those in bold refer to Tables

a angiotensin II receptor type 2 condensed α2‐macroglobulin 37 (AGTR2) 40 MCMDM‐1VWD 20 αIIbβ3 integrin receptor in ANKD26 mutations 144 evaluation of menstrual platelet antenatal diagnosis see blood loss 23–4 membrane 134 prenatal diagnosis International Society on ε‐aminocaproic acid 109 (PND) Thrombosis and abnormal uterine anterior spinal artery Haemostasis Bleeding (AUB) 30, 40 syndrome 196 Assessment Tool 21 AUB‐C 37 antiepileptics 216 molecular and clinical abscess, epidural 196 antifibrinolytics 72, 77, 104 markers for diagnosis acquired von Willebrand in factor XI deficiency 109 and management of syndrome (aVWS) 86 in heavy menstrual Type 1 VWD activated partial bleeding 55 (MCMDM‐1VWD) thromboplastin time in menorrhagia 127 14–20 (APTT) 5, 75, in VWD 91 Pediatric Bleeding 103, 207 antithrombin 110 Questionnaire (PBQ) in FXI deficiency 122 arachidonic acid 142 20–1 in FVII deficiency 122 aromatase 33 Self‐BAT 21–2 in VWD 88 aspirin 147 Self‐PBQ 21–2 acupuncture 192 atherosclerosis 90 Vicenza Bleeding ADAMTS13 83, 84 b Questionnaire and adenosine diphosphate (ADP) Score 14 Bernard–Soulier syndrome 142 Vincenza‐based, evolution (BSS) 21, 50, 145, adrenomedullin 39, 40 of 13–14, 14, 15–19 147, 214, 215 afibrinogenemia 9, 119, 121, women’s studies using COPYRIGHTEDbipolar radiofrequency MATERIAL 126, 127, 169, 217 Vincenza‐based ablation 57 alkaline hematin test 23, 120 bleeding tools 22–3 Bleeders Not Yet Defined alpha granule release young women’s hematology (NYD) 25 assessment 142 and bleeding 25 Bleeders of Unknown Cause amniocentesis 128, 144, 170, breastfeeding 90 (BUC) 25 172–4, 173, 209 broad ligament bleeding assessment tools third‐trimester 173–4 59 (BATs) 13–26, 48, 159 in VWD 90 butorphanol 193 clinical utility of 25–6

Inherited Bleeding Disorders in Women, Second Edition. Edited by Rezan A. Kadir, Paula D. James, and Christine A. Lee. © 2019 John Wiley & Sons, Ltd. Published 2019 by John Wiley & Sons, Ltd.

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c combined hormonal corpus luteum, bleeding carbonic anhydrase 9 36 contraceptives (COCs) from 6 Cardiff Penthrane factor XI deficiency and 104 cryoablation 57 inhaler 192 in heavy menstrual cryoprecipitate 124 cascade model 4 bleeding 53, 58 cumulus oophorus 32 cell‐based model of in menorrhagia 127 hemostasis 4–5, 4 combined oral contraceptive d cell‐free fetal DNA (cffDNA) pill (COCP) 9, 53, 147 Danazol 54 in maternal in VWD menorrhagia 92 DDAVP 70, 76, 109, 146 circulation 177 combined spinal‐epidural in heavy menstrual central nervous system (CNS) (CSE) 192, 195 bleeding 55–6, 58 bleeding 136 Condensed MCMDM‐1VWD in inherited platelet cephalohematoma 209, 211 Bleeding defects 144 cephalosporins 216 Questionnaire 22, 26 nasal spray 58 cervical cerclage 128 congenital amegakaryocytic in VWD type 2 and cervical lesions 123 thrombocytopenia type 3 53 cervicitis 123 (CAMT) 145, 214 deamino‐8‐arginine cesarean section in factor XI congenital bleeding disorders vasopressin see DDAVP deficiency 108 detection 209–13 decidualization 34, 35 CHAMP FVIII database 153 newborns presenting dense granule release CHBMP database 153 with bleeding assessment 142 chemokines 35 symptoms 211–13 dental surgery 146–7 C 35 newborns with positive depot medroxyprogesterone CC 35 family history of acetate (DMPA) 54 CCL11 35 bleeding desmopressin 55–6, 75, 93, CCL2 35 disorder 209–11 104, 109, 221 CX3C 35 optimal mode of in factor XI deficiency 110 CXC 35 delivery 210–11 in heavy menstrual CXCL10 35 prenatal bleeding 55–6 CXCL8 35, 36 diagnosis 209–10 in hemophilia A 110 chorionic villus sampling developmental in menorrhagia 127 (CVS) 74, 128, 144, hemostasis 205–6 for menorrhagia in women 170, 171–2, 209 diagnostic algorithm 206 with VWD 92 in VWD 90 laboratory hemostatic in VWD 90, 110 circumcision evaluation 208–9 see also DDAVP ® factor VI deficiency 111 management desogestrel (Cerazette ) 54 in newborn 213 congenital bleeding digital PCR (dPCR) 179 Clauss fibrinogen 9 disorders 218–21, 220 disseminated intravascular clotting factors when newborn is coagulation in heavy menstrual bleeding 219–21 (DIC) 109, 214 bleeding 56 presentation 213–18 double uterotonics 77 in menorrhagia 127 primary hemostasis 206–7 droplet digitial PCR coagulation disorders 215–18 secondary (ddPCR) 179 coagulation factors, hemostasis 207–8 Duchenne muscular rare 216–18, 217 connective tissue growth dystrophy 181 coagulation pathways 3, 37 factor (CTGF) 40 dural puncture 195 collagen 3, 142 consanguineous dyschezia 59 collagens I, III, IV and marriage 231 dysfibrinogenemia 9, 121, VI 35 cordocentesis 170, 174–6, 175 122, 127

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dysmenorrhea 59 f factor XI deficiency (FXI) 1, dyspareunia, deep 59 factor II see prothrombin 10, 45, 59, 101–11, 124, factor V 3 127, 128 e factor V deficiency 5, 10, 50, in Ashkenazi Jews 211 Ehlers‐‐Danlos syndrome 7 117, 122, 126, 169 bleeding manifestations and Eltrombopag 147 in newborn 217–18 diagnosis 103–4 embryo culture 181 postpartum hemorrhage coagulation cascade 102 endometrial ablation in 121 gynecological in heavy menstrual prevalence 117 surgery 104–5 bleeding 56–7 factor V + factor VIII deficiency, heavy menstrual in menorrhagia 127 combined 10, 66, 117, bleeding 103–4 endometrial hyperplasia 60 122, 124, 169 hysterectomy 104 endometrial polyps 123 prevalence 117 incidence and endometriosis 6, 59–60, 121 factor Va 5 inheritance 101–3 link between IBM and 60 factor VII 3, 9, 37, 101 inheritance 169 endothelin‐1 (ET‐1) 37 postpartum hemorrhage neonatal bleeding 111–12 enflurane 193 in 122 in newborn 218 Entonox 192 factor VII deficiency 5, 10, neuroaxial analgesia and enzyme‐linked 50, 117, 169 anesthesia 108 immunosorbent assay in newborn 218 postpartum hemorrhage (ELISA) 9, 142 prenatal diagnosis 180 and 122 epidural 192 prevalence 117 postpartum period, epidural abscess 196 severe 126, 128 management of 108–9 epidural hematoma 196, 199 factor VIIa 101 and 105–8, epinephrine 142 factor VIII (FVIII:C) 3, 9, 84 106, 192 episiotomy 107 in hemophilia, neonate antenatal estradiol 30 l, 33 76 management 105 estrogen receptor (ER) 33 in pregnancy 192 bleeding tests 105–7 ERα 33, 34 factor VIII deficiency 153 bleeding ERβ 33 in newborn 219 phenotype 105–6 ethinyl estradiol, low‐dose 53 factor VIIIa 5 blood group 106 European Association for factor IX 192 factor XI level 106–7 Haemophilia and in hemophilia, neonate fibrin density Allied Disorders 76 network 107 (EAHAD) international in pregnancy 192 global testof database 66, 153 factor IX deficiency 5, 153, hemostasis 107 European Molecular and 169, 219 labor delivery 107–8 Clinical Markers for see also hemophilia B in mild/moderate FXI the Diagnosis and factor IXa 4 deficiency 108 Management of Type 1 factor X 9, 101 requiring cesarean von Willebrand Disease factor X deficiency 10, 50, section 108 (MCMDM‐1VWD) 122, 124, 169 in severe FXI study 20 in newborn 218 deficiency 107–8 European Network of the Rare prenatal diagnosis 180 peripartum Bleeding Disorders prevalence 117 management 105–7 (EN‐RBD) 117 severe 126, 128 prevalence 117, 118 extracranial hemorrhage (ECH) factor XI 104, 106–7 structure and function in in newborn 209, concentrates 109 coagulation 101, 102 211–12 in pregnancy 192 treatment 109–11

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factor XI replacement confirmation of hormonal therapy 109–10 diagnosis 168 treatments 51–5 factor XIa 3, 5 inheritance 168 non‐hormonal factor XIII 4, 5, 37 reproductive treatments 55–6 factor XIII deficiency 5, 10, options 169, 70 surgical interventions 56–8 50, 59, 117, 124, 127, genome editing 147 ovulation bleeding 59 128, 169 Glanzmann as predictor for bleeding in 121 thrombasthenia 21, disorders 46–7 in newborn 218, 219 50, 137, 144–5, 146, screening for bleeding PPH in 121 147, 214, 215 disorders in 47–8 phenotype 122 in French gypsies 211 hemarthroses 136, 213 prenatal diagnosis 180 glycoprotein Ib 3 hematoma prevalence 117 glycoprotein Ib‐IX‐V broad ligament 59 severe 126 complex 134 epidural 196, 199 fentanyl 193 glycoprotein IIb/IIIa platelet muscle 136 fibrin 4 receptor 3 in newborn 213 fibrinogen 4, 9 glycoprotein VI 3, 134 retroperitoneal 59 in pregnancy 127 gonadotrophin‐releasing spinal 196, 199 fibrinogen deficiency 10, 50, hormone 30 subgaleal 209, 211 124, 128 agonist in heavy menstrual hematopoietic stem cell miscarriage in 121 bleeding 54 transplantation in in newborn 217 pulse generator 31 inherited platelet phenotype 122 gonadotrophins 30 defects 145 prevalence 117 gray platelet syndrome hemoperitoneum 59 thrombin time (TT) 214 hemophilia 5 in 122 growth factor binding prenatal diagnosis of 177– fibrinolysis 4, 36–7 protein‐1 35 80, 178 fibroids 6, 54, 121 gynecology 45–61 hemophilia A (HA) 5, 22, 57, factor XI deficiency 104 117, 192, 215 submucosal 123 h desmopressin in 110 uterine 50, 60 heat shock proteins 1A and FVIII:C levels 154, 155 fibronectin 3 1B 40 genetic analysis 160–2 flow cytometry 142, 143 heavy menstrual bleeding guidelines 164 fluorescent in situ (HMB) 1, 37, 40, 45, 47, inheritance 168 hybridization (FISH) 47, 48–9, 119–21, 20 phenotypic analysis 154–6 172, 181 algorithm of prevalence 153, 154 follicle aromatase 30 management 51 severity 154 free fetal DNA (ffDNA) diagnosis 45–6 variant types 160 170 endometriosis 59–60 variants 153 free fluid ablation 57 IBD and 49–50 hemophilia A, carriers fresh frozen plasma (FFP) 104, management 51–2 of 65–79, 192 109, 110, 124 incidence 29 bleeding disorders FSH 30 investigations 50–1 in 68–73 management of acute FVIII or FIX g episodes 58 deficiency 68–9 gene therapy in Wiskott– COCs 58 FVIII:C/FIX:C levels and Aldrich syndrome 147 LNG‐IUS 58 bleeding scores in 71 genetic counselling 167–70 nasal DDAVP spray 58 imprecise designation with clinical phenotype 169 medical treatment 51–8 low factor level 70

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low FVIII or FIX imprecise designation Hermansky–Pudlak levels 69 with low factor syndrome 145 menorrhagia 72 level 70 high forceps 210 prevalence 70 low FVIII or FIX hormone replacement therapy protection from levels 69 (HRT) 9, 60 thrombosis 73 menorrhagia 72 hyperplasia 6, 121 terminology 69–70 prevalence 70 hypofibrinogenemia 9, 121, treatment of bleeding protection from 127, 217 episodes 70–1 thrombosis 73 PPH in 122 type of bleeding terminology 69–70 hyponatremia 90 episodes 72 treatment of bleeding care of carriers 77–9 episodes 70–1 169 delayed genetic diagnosis/ type of bleeding hypothalamic‐pituitaryovarian carriership 68 episodes 72 axis 31, 32 F8 or F9 gene mutation 66 care of carriers 77–9 hypoxia inducible factor 1α genetic vs coagulation delayed genetic diagnosis/ (HIF‐1α) 36, 39 testing 66–7 carriership 68 hysterectomy inheritance 65, 66 F8 or F9 gene mutation 66 in heavy menstrual pregnancy in 73–7 genetic vs coagulation bleeding 57–8 preimplantation genetic testing 66–7 in menorrhagia 127 diagnosis (PGD) 67 inheritance 65, 66, 168 hysteroscopy 50, 57 prenatal diagnosis (PND) 67 pregnancy in 73–7 quality of life 73 preimplantation genetic i screening of 65–6 diagnosis (PGD) 67 immune thrombocytopenias timing of genetic prenatal diagnosis (PND) 67 (ITPs) 214 testing 67–8 quality of life of carriers 73 immunoglobulin 109 vignettes 78–9 screening of 65–6 in vitro fertilization hemophilia B 5, 117, 215, timing of genetic (IVF) 181, 210 222 testing 67–8 inherited bleeding in Amish vignettes 78–9 disorder(IBDs) 1–10, communities 211 hemorrhage 45, 48 FIX:C levels 156 extracranial (ECH) 209 bleeding, clinical genetic analysis 162–3 intracranial (ICH) 68, presentation of 5–6 guidelines 164 76–7, 209, 212, 212 diagnosis 6 inheritance 65, 66, 168 see also postpartum heavy menstrual bleeding phenotypic analysis 156–7 hemorrhage and 49–50, 61 in pregnancy 192 hemostasis history 6–7, 7 prevalence 153, 154 cell‐based model of 4–5, 4 medication history 6 severity 154 defects of 5 pastmedical historyand variant types 160 fibrinolysis 4 family history 6 variants 153 normal 1–3, 2 investigations 7–10 hemophilia B, carriers primary 3 hemostatic assays of 65–79 secondary 3–4 laboratory assessment of bleeding disorders hemostatic function platelet hematological and in 68–73 function analyzer coagulation system 7, FVIII or FIX (PFA‐200) 89 8–9 deficiency 68–9 hemostatic risk 191–2 preanalytical factors in FVIII:C/FIX:C levels and heparin 109 coagulation bleeding scores in 71 hepatitis A and B viruses 91 testing 9–10

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investigations (Contd.) international normalized ratio laminin 3 specialized tests of (INR) 207 Lebanon coagulation 10 intracranial hemorrhage (ICH) inherited bleeding disorders physical assessment 6–7 68, 76–7 in 225 prevalence 13 in newborn 209, 211–12, inherited factor VII phenotypic analysis 212 deficiency 230–1, 232 of 157–60 intracytoplasmic sperm type 3 VWD 229–30, 231 inherited platelet defects injection (ICSI) 181 leptin 31 (IPDs) 10, 133–48, intramuscular analgesia levonorgestrel 53 213–15 193 levonorgestrel intrauterine case histories 147–8 intravenous opioid device (IUD) 159 causative gene 138–41, 143 administration 193 in menorrhagia 127 future directions 147 iron deficiency 48 levonorgestrel intrauterine investigation 137–43 iron deficiency 50 system (IUS) management 144–7 isoflurane 193 factor XI deficiency dental surgery 146–7 isoniazid 216 and 104 menorrhagia 147 in menorrhagia 147 pregnancy and k levonorgestrel‐releasing delivery 145–6 kisspeptin 31 intrauterine device 92 supportive levonorgestrel‐releasing management 147 l intrauterine system surgery 146–7 labor pain 191 (LNG‐IUS) in heavy treatment options 144–5 non‐pharmcological menstrual normal platelet management 192 bleeding 51–2, 52, 58 function 133–4 pharmacological light transmission aggregometry prenatal diagnosis 143–4 management 192– (LTA) 142, 143 presentation 136–7 201 loss of resistance inherited thrombocytopenias general anesthesia 201 technique 194 134, 136 inhalation low molecular weight heparin related to GATA‐1 analgesia 192–3 in VWD 94 mutations 314 opioid analgesia 193–4 lumiaggregometry 21, 142 integrin receptor α2β1 134 postoperative pain 201 luminometry 142 interleukin (IL) regional (neuraxial) lupus anticoagulant 9 IL‐6 35 analgesia and lyonization 68 IL‐15 35 anesthesia 194–9 intermenstrual bleeding, coagulopathy and risk of m irregular 60 neurological macrophages 36 International Society on complications 195–6 macrothrombo Thrombosis and guidance for use in cytopenias 214 Haemostasis (ISTH) IBDs 198–9, 198, 200 Malaysia 232 Scientific and Standardization regional blockade in inherited bleeding disorders Committee IBD 197–8 in 225–9 (SSC) 13–14 risks and complications 195 Glanzmann International Society on techniques of blockade thrombasthenia 228, Thrombosis and 194–5 231 Haemostasis Bleeding regional block techniques, VWD type 2 227–8, Assessment Tool other 199–201 231, 232 (ISTH‐BAT) 6, 21, paracervical block 199–200 massively parallel sequencing 22, 25, 137 pudendal block 200–1 (MPS) 179

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matrix metalloproteinases molecular and clinical markers hemorrhagic 6, 59, 61, 121 (MMPs) 36, 37 for the diagnosis and ruptured 61, 137 MCMDM‐1VWD Bleeding management of ovulation bleeding 59 Questionnaire Type 1 VWD oxytocin 195 20, 26 (MCMDM‐1VWD) mean platelet volume 14–20 p (MPV) 134 condensed 20 pain, definition 191 Mediterranean microthrombo monogenic diseases (MGD) Pakistan, inherited bleeding cytopenia 214 181 disorders in, type 3 medroxyprogesterone morphine 193 VWD 225–7, 231, 232 acetate 58 mucocutaneous type PALMCOEIN acronym 30 megakaryocytes 133 bleeding 209 patient‐controlled analgesia menarche 31 muscle 136 (PCA) 193 menorrhagia 5–6, 26, 70, musculoskeletal patient‐controlled epidural 119–21, 120 bleeding 209 analgesia (PCEA) 194 differential MYH9‐related disorder 137, Pediatric Bleeding diagnosis 123–4 142, 214 Questionnaire IPDs presenting with 136 MYH9‐related (PBQ) 20–1, 25, 26 management 147 thrombocytopenia Self 21–2 vWD and 87–8, 91, 92 147 pelvic pain, chronic 59 menstrual blood loss (MBL) petechiae 209 45–6 n pethidine 193 ® menstrual cup (Mooncup ) 46 naloxone 193 PGE2 37 menstruation 29–40 natural killer (NK) cells 34 PGF2α 36, 37 control of bleeding 37–8 neonatal alloimmune Phillipp screening tool endometrial thrombocytopenia 24, 25 regeneration 40 137, 146 Pictorial Blood Assessment endometrial repair 38–9 next‐generation sequencing Chart (PBAC) 23–5, endometrium 33–4 (NGS) 10, 143 23–4, 46, 104, 120, 136, neuroendocrine NF‐κB 36 159, 232 hormones 30–1 nitrous oxide 193 plasmin 4, 37 normal menstruation non‐invasive prenatal diagnosis plasminogen 37, 207 29–30 (NIPD) 176–7 plasminogen activator duration 30 non‐resectoscopic inhibitor (PAI) 37 volume of blood loss 30 endometrial ablation platelet activation 3 ovarian follicle development (NREA) 56 platelet activation status 134 and endocrine non‐steroidal anti‐ platelet adhesion 3 function 31–3 inflammatory drugs platelet aggregation 3, 134 physiology of 29–31 (NSAIDs) 55, 72, 93, 147 platelet beta 1 integrin 3 proliferative phase 40 norethisterone 58 platelet count 134 ® secretory phase 34–5 NovoSeven in inherited platelet formation 134, 135 meperidine 193, 194 platelet platelet function analyzer meptazinol 193 defects 144–5 (PFA)‐100 mesenchymal‐to‐epithelial platelet function disorders 5, transition (MET) 39 o 45, 103, 214–15 methoxyflurane 192 obstetric cholestasis 196 platelet granules 134 121 ocular albinism 7 alpha 134 Mittelschmerz (midcycle pain) oral progesterone tablets 147 dense 134 59 ovarian cyst platelet plug formation 3

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platelet transfusion in inherited carrier attitudes global distribution 118 platelet defects 145 towards 183–4 gynecological and obstetrical Poiseuille’s equation 37, 38 of factor VII, factor X and manifestations polyps 6, 121 factor XIII 119–22 endometrial 123 deficiencies 180 laboratory diagnosis 122–3 factor XI deficiency 104 of hemophilia 177–80, phenotype 122 uterine 50, 60 178 molecular postcircumcision invasive 170–6 diagnosis 122–3 bleeding 209 non‐invasive prenatal diagnosis of 180–1 postmenopausal bleeding (NIPD) 176–7 treatment 124–8, 125 (PMB) 60 of rare bleeding clotting factor postpartum hemorrhage disorders 180–1 replacement 124–7 (PPH) 6, 70, 77, 87–8, of VWD 180 management of pregnancy 103, 120, 121–2 primary hemostasis 83 and 127–8 in factor XI deficiency 105 prion diseases 91 treatment of pre‐eclampsia 90, 196 progesterone 30, 34, 35–6 menorrhagia 127 pregnancy in hemophila progesterone receptor (PR) 33 receiver operator curve (ROC) carriers 73–7 PRA 34 20–1 antenatal management73–5 PRB 34 recombinant factor VIIa changes of factor progestin‐only “mini‐pill” 54 (rFVIIa) 109, 110–11, levels 74–5 progestogens in heavy 126 preimplantation genetic menstrual in inherited platelet diagnosis (PGD) 74 bleeding 53–4, 58 defects 144–5 prenatal diagnosis 73–4 prostaglandin E2 35 recombinant factor perinatal management 75–7 prostaglandin F2α 35 XIIIa 104, 126 cranial imaging 76 protease tissue plasminogen recombinant VWF intracranial hemorrhage activator (tPA) 4 concentrate 91 and relation to mode of prothrombin 5, 9, 37 relative haplotype dosage delivery 76–7 prothrombin complex (RHDO) 179–80 mother ‐ regional concentrates (PCCs), relative mutation dosage block 75 124, 126 (RMD) 179 neonate 75–6 prothrombin deficiency 10, remifentanil 193, 194, 202 postpartum 50, 126 resectoscope 56 hemorrhage 77 in newborn 217 resectoscopic endometrial preimplantation embryo PPH in 122 ablation (REA) 56–7 selection (PIES) 210 prevalence 117 restriction fragment length preimplantation genetic prothrombin time (PT) 5, polymorphisms diagnosis 181–3 207 (RFLPs) 177 carrier attitudes 183–4 puberty see menarche retrograde menstruation experiences and purpura 209 theory 59 psychological retroperitoneal effects 184–5 r hematoma 59 towards prenatal rare bleeding disorders (RBDs) rifampin 216 diagnosis 183–4 117–28 ristocetin 142, 215 termination of classification 126 ristocetin co‐factor assay, pregnancy 184, 185 clinical symptoms 118–19 VWF:RCo 89 Premarin 58 differential diagnosis in rotational thromboelastometry prenatal diagnosis menorrhagia 123–4 (ROTEM) 10 (PND) 167–85 gender 118 RUNX1 mutation 144

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s in VWD 91 Vicenza Bleeding Self‐BAT 21–2 transcervical endometrial Questionnaire and Self‐PBQ 21–2 resection 57 Score 14 sevoflurane 193 transdermal contraceptive vitamin K 76, 146 solvent detergent (SD) FFP 110 patches 53 at birth 211, 212–13 spinal hematoma 196, 199 transforming growth factor β2 metabolism 122 subgaleal hematoma 209, 211 (TGFβ2) 40 bleeding submucosal fibroids 123 Tuohy’s curved bevel (VKDB) in Swyer syndrome 68 needle 194, 195, 196 infancy 216 Turner syndrome 68, 156 vitamin K‐dependent t coagulation factor termination of u deficiency (VKCFD) pregnancy 184, 185 Ulipristal acetate (UPA) 117, 122 ® hemophilia A and B (Esmya ) in heavy von Willebrand disease and 73–4 menstrual (VWD) 1, 5, 6, 9, 37, thermal balloon ablation 57 bleeding 54–5 45, 47, 57, 83–96, 103, thrombin 3, 4, 5, 37 ultra‐large (ULVWF) VWF 117, 142, 215–16 thrombin activatable multimers 207 cases 94–6 fibrinolysis inhibitor ultrasound examination, classification and (TAFI) 5, 101 fetal 170 inheritance 85, 86 thrombin generation test umbilical stump clinical presentation 87 (TGT) 107 bleeding 212 diagnosis 10, 13, 14, 88–9 thrombocytopenia 37, 146, ureteric silk catheters 194 inheritance 168–9 213–14 urokinase‐like plasminogen VWF:Ag levels 154 inheriteds 134, 136, 314 activator (uPA) 4 family testing 89–90 thrombocytopenia with absent uterine fibroid 50, 60 genetic analysis 163–4 radii (TAR) 214 uterine natural killer (uNK) genetic analysis 89–90 thromboelastography cells 34–5 guidelines 164 (TEG) 10, 107 uterine polyps 50, 60 gynecological bleeding, thromboelastometry uterine tony 121 management of 91 (ROTEM) 107 history 83 tissue factor (TF) 3, 37, 101 v inheritance 168–9 tissue factor pathway inhibitor vaccines 212–13 laboratory testing 88–9 (TFPI) 101 vacuum extraction 210 management 90–1 tissue inhibitors of vaginal rings 53 menorrhagia and 87–8, metalloproteinases vaginal tears 107, 123 91, 92 (TIMPSs) 38 valerate and dienogest (E2V/ neonatal management 94 tissue plasminogen activator DNG) contraceptive obstetric (u‐PA) 37 pill 53 management 92–4 tramadol 193 variant Creutz‐Jakob antenatal tranexamic acid 6, 93, 94, disease 91 management 92–3 108, 109, 127, 147, 197 vascular endothelial growth intrapartum factor XI deficiency 104 factor (VEGF) 36 management 93 in heavy menstrual VEGFR‐1 (flt‐1) 39 postpartum bleeding 55 VEGFR‐2 (KDR) 39 management 93–4 in inherited platelet VEGR 39, 40 pre‐pregnancy defects 144 venepuncture 212–13 management 92 for menorrhagia in vessel radius, effect on on phenotypic analysis 157, VWD 92 blood flow 37, 38 158

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von Willebrand disease inheritance 169 von Willebrand syndrome, (VWD) (Contd.) in pregnancy 192 acquired (aVWS) 86 postpartum hemorrhage variance 153 von Willebrand factor and 87–8 von Willebrand factor (VWF) pseudogene prenatal diagnosis 89–90, 3, 5, 9, 37, 47, 103 (VWFP1) 153 180 deficiency 153 VWF see von Willebrand platelet‐type VWD 214 levels and prevalence of factor (VWF) type 1 85, 87–8, 89, 90, 92, VWD 84 w 93, 153 menorrhagia and warfarin 216 normal clotting factor 87–8 Willebrand disease (VWD) 66 levels 192 menstrual cycle, pregnancy Wiskott–Aldrich syndrome type 2 48, 53, 85, 89, 90, and 84–5 (WAS) 145, 147, 214 92, 93, 153 postpartum hemorrhage World Federation of type 2A 169 and 87–8 Hemophilia type 2B 169, 214 structure and (WFH) 117 type 2M 154, 169 function 83–4 type 2N 154, 169 von Willebrand factor antigen x type 3 48, 53, 85–7, 89–90, (VWF:Ag. X‐linked thrombo 92, 93–4, 153, 219 VWF:Ac) 192 cytopenia 147

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