Women and von Willebrand disease: Controversies in diagnosis and management

Seminars in Thrombosis and Hemostasis

Volumn 32, Issue 6, 2006, Pages 605-615

  Kadir, Rezan A ^a   Chi, Claudia ^a  

^a ROYAL FREE HOSPITAL   (United Kingdom)

Author keywords

Diagnosis; Management; Menorrhagia; Von Willebrand disease; Women

Indexed keywords

ANTIFIBRINOLYTIC AGENT; BLOOD CLOTTING FACTOR 8; DANAZOL; DESMOPRESSIN; ESTRADIOL; ESTROGEN; ETHINYLESTRADIOL; FLURBIPROFEN; GESTAGEN; GONADORELIN AGONIST; LEVONORGESTREL; MEFENAMIC ACID; NONSTEROID ANTIINFLAMMATORY AGENT; ORAL CONTRACEPTIVE AGENT; RISTOCETIN; TRANEXAMIC ACID; VON WILLEBRAND FACTOR;

ADULT; AMENORRHEA; BLEEDING DISORDER; BLOATING; BLOOD CLOTTING FACTOR DEFICIENCY; BREAKTHROUGH BLEEDING; BREAST TENDERNESS; CONTROLLED STUDY; DEPRESSION; DIARRHEA; DRUG DELIVERY SYSTEM; DRUG EFFICACY; DYSMENORRHEA; ENDOMETRIUM ABLATION; FATIGUE; FEMALE; FLUID RETENTION; FLUSHING; GYNECOLOGIC DISEASE; HEADACHE; HUMAN; HYPERTENSION; HYPONATREMIA; HYSTERECTOMY; LABORATORY TEST; LIVER TUMOR; MAJOR CLINICAL STUDY; MENORRHAGIA; MENSTRUAL CYCLE; MIGRAINE; MOOD CHANGE; NAUSEA; PREMENSTRUAL SYNDROME; PREVALENCE; PRIORITY JOURNAL; REVIEW; SCREENING TEST; SIDE EFFECT; SKIN TOXICITY; TACHYCARDIA; THROMBOSIS; TREATMENT OUTCOME; VOMITING; VON WILLEBRAND DISEASE; WEIGHT GAIN;

ADOLESCENT; ADULT; ANTIFIBRINOLYTIC AGENTS; CONTRACEPTIVES, ORAL, SYNTHETIC; DEAMINO ARGININE VASOPRESSIN; ENDOMETRIUM; FEMALE; GENITAL DISEASES, FEMALE; HUMANS; LEVONORGESTREL; MENORRHAGIA; MIDDLE AGED; QUALITY OF LIFE; TRANEXAMIC ACID; VON WILLEBRAND DISEASE; VON WILLEBRAND DISEASES;

EID: 33748997650     PISSN: 00946176     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-2006-949665     Document Type: Review

References (92)

1. Federici AB, Mannucci PM. Actual management of von Willebrand disease: first report on 880 cases of the Italian registry of VWD. Blood 1997;90:33a (abst)
2. Foster PA. The reproductive health of women with von Willebrand disease unresponsive to DDAVP: results of an international survey. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 1995;74:784-790
3. Ragni MV, Bontempo FA, Hassett AC. von Willebrand disease and bleeding in women. Haemophilia 1999;5:313-317
4. Kadir RA, Economides DL, Sabin CA, Pollard D, Lee CA. Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders. Haemophilia 1999;5:40-48
5. Kouides PA, Phatak PD, Burkart P, et al. Gynaecological and obstetrical morbidity in women with type I von Willebrand disease: results of a patient survey. Haemophilia 2000;6:643-648
6. Rozeik C, Scharrer I. Gynecological disorders and psychological problems in 184 women with von Willebrand disease. Haemophilia 1998;4:293
7. Kirtava A, Drews C, Lally C, Dilley A, Evatt B. Medical, reproductive and psychosocial experiences of women diagnosed with von Willebrand's disease receiving care in haemophilia treatment centres: a case-control study. Haemophilia 2003;9:292-297
8. James AH. More than menorrhagia: a review of the obstetric and gynaecological manifestations of bleeding disorders. Haemophilia 2005;11:295-307
9. Barr RD, Sek J, Horsman J, et al. Health status and health-related quality of life associated with von Willebrand disease. Am J Hematol 2003;73:108-114
10. Barr RD, Saleh M, Furlong W, et al. Health status and health-related quality of life associated with hemophilia. Am J Hematol 2002;71:152-160
11. Whitton AC, Rhydderch H, Furlong W, Feeny D, Barr RD. Self-reported comprehensive health status of adult brain tumor patients using the Health Utilities Index. Cancer 1997;80:258-265
12. Kadir RA, Sabin C, Pollard D, Lee C, Economides DL. Quality of life during menstruation in patients with inherited bleeding disorders. Haemophilia 1998;4:836-841
13. Bruner AB, Joffe A, Duggan AK, Casella IF, Brandt J. Randomised study of cognitive effects of iron supplementation in non-anaemic iron-deficient adolescent girls. Lancet 1996;348:992-996
14. Patterson AJ, Brown WJ, Roberts DCK. Dietary and supplement treatment of iron deficiency results in improvements in general health and fatigue in Australian women of childbearing age. J Am Coll Nutr 2001;20:337-342
15. Shankar M, Lee CA, Sabin CA, Economides DL, Kadir RA. von Willebrand disease in women with menorrhagia: a systematic review. BJOG 2004;111:734-740
16. James A, Matchar DB, Myers ER. Testing for von Willebrand disease in women with menorrhagia: a systematic review. Obstet Gynecol 2004;104:381-388
17. Philipp CS, Faiz A, Dowling N, et al. Age and the prevalence of bleeding disorders in women with menorrhagia. Obstet Gynecol 2005;105:61-66
18. American College of Obstetricians and Gynecologists Committee Opinion No 263. von Willebrand's disease in gynecologic practice. Obstet Gynecol 2001;98:1185-1186
19. Dilley A, Drews C, Lally C, Austin H, Barnhart E, Evatt B. A survey of gynecologists concerning menorrhagia: perceptions of bleeding disorders as a possible cause. J Womens Health Gend Based Med 2002;11:39-44
20. Chi C, Shiltagh N, Kingman CEC, Economides DL, Lee CA, Kadir RA. Identification and management of women with inherited bleeding disorders: a survey of obstetricians and gynaecologists in the United Kingdom. Haemophilia 2006;12:405-412
21. Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Frequency of inherited bleeding disorders in women with menorrhagia. Lancet 1998;351:485-489
22. Quiroga T, Goycoolea M, Munoz B, et al. Template bleeding time and PFA-100 have low sensitivity to screen patients with hereditary mucocutaneous hemorrhages: comparative study in 148 patients. J Thromb Haemost 2004;2:892-898
23. James AH, Lukes AS, Brancazio LR, Thames E, Ortel TL. Use of a new platelet function analyzer to detect von Willebrand disease in women with menorrhagia. Am J Obstet Gynecol 2004;191:449-455
24. Kouides PA, Conard J, Peyvandi F, Lukes A, Kadir R. Hemostasis and menstruation: appropriate investigation for underlying disorders of hemostasis in women with excessive menstrual bleeding. Fertil Steril 2005;84:1345-1351
25. Rodeghiero F, Castaman G, Tosetto A, et al. The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study. J Thromb Haemost 2005;3:2619-2626
26. Fraser IS, McCarron G, Markham R. A preliminary study of factors influencing perception of menstrual blood loss volume. Am J Obstet Gynecol 1984;149:788-793
27. Chimbira TH, Anderson ABM, Turnbull AC. Relation between measured menstrual blood loss and patient's subjective assessment of loss, duration of bleeding, number of sanitary towels used, uterine weight and endometrial surface area. Br J Obstet Gynaecol 1980;87:603-609
28. Hallberg L, Hogdahl AM, Nilsson L, Rybo G. Menstrual blood loss-a population study. Variation at different ages and attempts to define normality. Acta Obstet Gynecol Scand 1966;45:320-351
29. Hallberg L, Nilsson L. Determination of menstrual blood loss. Scand J Clin Lab Invest 1964;16:244-248
30. Warner PE, Critchley HO, Lumsden MA, Campbell-Brown M, Douglas A, Murray GD. Menorrhagia II: is the 80-mL blood loss criterion useful in management of complaint of menorrhagia? Am J Obstet Gynecol 2004;190:1224-1229
31. Warner PE, Critchley HO, Lumsden MA, Campbell-Brown M, Douglas A, Murray GD. Menorrhagia I: measured blood loss, clinical features, and outcome in women with heavy periods: a survey with follow-up data. Am J Obstet Gynecol 2004;190:1216-1223
32. Higham JM, O'Brien PM, Shaw RW. Assessment of menstrual blood loss using a pictorial chart. Br J Obstet Gynaecol 1990;97:734-739
33. Janssen CA, Scholten PC, Heintz AP. A simple visual assessment technique to discriminate between menorrhagia and normal menstrual blood loss. Obstet Gynecol 1995;85:977-982
34. Kirtava A, Crudder S, Dilley A, Lally C, Evatt B. Trends in clinical management of women with von Willebrand disease: a survey of 75 women enrolled in haemophilia treatment centres in the United States. Haemophilia 2004;10:158-161
35. Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Variations in coagulation factors in women: effects of age, ethnicity, menstrual cycle and combined oral contraceptive. Thromb Haemost 1999;82:1456-1461
36. Miller CH, Dilley A, Richardson L, Hooper WC, Evatt L. Population differences in von Willebrand factor levels affect the diagnosis of von Willebrand disease in African-American women. Am J Hematol 2001;67:125-129
37. Sukhu K, Poovalingam V, Mahomed R, Giangrande PLF. Ethnic variation in von Willebrand factor levels can influence the diagnosis of von Willebrand disease. Clin Lab Haematol 2003;25:247-249
38. Dilley A, Drews C, Miller C, et al. von Willebrand disease and other inherited bleeding disorders in women with diagnosed menorrhagia. Obstet Gynecol 2001;97:630-636
39. Gill JC, Endres-Brooks J, Bauer PJ, Marks WJ, Montgomery RR. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987;69:1691-1695
40. Nitu-Whalley IC, Lee CA, Griffioen A, Jenkins PV, Pasi KJ. Type 1 von Willebrand disease-a clinical retrospective study of the diagnosis, the influence of the ABO blood group and the role of the bleeding history. Br J Haematol 2000;108:259-264
41. Bauduer F, Ducout L. Is the assessment of von Willebrand disease prevalence an achievable challenge? The example of the French Basque Country where blood group O and factor XI deficiency are highly prevalent. J Thromb Haemost 2004;2:1724-1726
42. Sadler JE. Slippery criteria for von Willebrand disease type 1. J Thromb Haemost 2004;2:1720-1723
43. Blombäck M, Eneroth P, Andersson O, Anvret M. On laboratory problems in diagnosing mild von Willebrand's disease. Am J Hematol 1992;40:117-120
44. Mandalaki T, Louizou C, Dimitriadou C, Symeonidis P. Variations in factor VIII during the menstrual cycle in normal women. N Engl J Med 1980;302:1093-1094
45. Miller CH, Dilley AB, Drews C, Richardson L, Evatt B. Changes in von Willebrand factor and factor VIII levels during the menstrual cycle. Thromb Haemost 2002;87:1082-1083
46. Onundarson PT. Treatment of menorrhagia in von Willebrand's disease. Haemophilia 1999;5:76
47. Beller FK, Ebert C. Effects of oral contraceptives on blood coagulation. A review. Obstet Gynecol Surv 1985;40:425-436
48. Prasad RN, Koh SC, Viegas OA, Ratnam SS. Effects on haemostasis after two year use of low dose combined oral contraceptive with gestodene or levonorgestrol. Clin Appl Thromb Hemost 1998;5:60-70
49. Harris GM, Stendt CL, Vollenhoven BJ, Gan TE, Tipping PG. Decreased plasma tissue factor pathway inhibitor in women taking combined oral contraceptives. Am J Hematol 1999;60:175-180
50. Royal College of Obstetricians and Gynaecologists. The Initial Management of Menorrhagia-National Evidence-Based Clinical Guidelines. London: RCOG Press; 1998
51. Royal College of Obstetricians and Gynaecologists. The Management of Menorrhagia in Secondary Care-National Evidence-Based Clinical Guidelines. London: RCOG Press; 1999
52. Kadir RA, Lukes AS, Kouides PA, Fernandez H, Goudemand J. Management of excessive menstrual bleeding in women with hemostatic disorders. Fertil Steril 2005;84:1352-1359
53. Wellington K, Wagstaff AJ. Tranexamic acid: a review of its use in the management of menorrhagia. Drugs 2003;63:1417-1433
54. Bonnar T, Sheppard BL. Treatment of menorrhagia during menstruation: randomised controlled trial of ethamsylate, mefenamic acid, and tranexamic acid. BMJ 1996;313:579-582
55. Milsom I, Andersson K, Andersch B, Rybo G. A comparison of flurbiprofen, tranexamic acid, and a levonorgestrel-releasing intrauterine contraceptive device in the treatment of idiopathic menorrhagia. Am J Obstet Gynecol 1991;164:879-883
56. British Medical Association and the Royal Pharmaceutical Society of Great Britain. British National Formulary. London: British Medical Association and the Royal Pharmaceutical Society of Great Britain; 2004
57. Ong YL, Hull DR, Mayne EE. Menorrhagia in von Willebrand disease successfully treated with single daily dose tranexamic acid. Haemophilia 1998;4:63-65
58. Rybo G. Tranexamic acid therapy: effective treatment in heavy menstrual bleeding: clinical update on safety. Therapeutic Advances 1991;4:1-8
59. Callard GV, Litofsky FS, DeMerre LJ. Menstruation in women with normal or artificially controlled cycles. Fertil Steril 1966;17:684-688
60. Ramcharan S, Pellegrin FA, Ray RM, Hsu JP. The Walnut Creek Contraceptive Drug Study. A prospective study of the side effects of oral contraceptives. Volume III, an interim report: a comparison of disease occurrence leading to hospitalization or death in users and nonusers of oral contraceptives. J Reprod Med 1980;25:345-372
61. Nilsson L, Rybo G. Treatment of menorrhagia. Am J Obstet Gynecol 1971;110:713-720
62. Fraser IS, McCarron G. Randomized trial of 2 hormonal and 2 prostaglandin-inhibiting agents in women with a complaint of menorrhagia. Aust N Z J Obstet Gynaecol 1991;31:66-70
63. Foster PA. The reproductive health of women with von Willebrand Disease unresponsive to DDAVP: results of an international survey. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 1995;74:784-790
64. Sulak PJ, Cressman BE, Waldrop E, Holleman S, Kuehl TJ. Extending the duration of active oral contraceptive pills to manage hormone withdrawal symptoms. Obstet Gynecol 1997;89:179-183
65. Vercellini P, De Giorgi O, Mosconi P, Stellato G, Vicentini S, Crosignani PG. Cyproterone acetate versus a continuous monophasic oral contraceptive in the treatment of recurrent pelvic pain after conservative surgery for symptomatic endometriosis. Fertil Steril 2002;77:52-61
66. Miller L, Notter KM. Menstrual reduction with extended use of combination oral contraceptive pills: randomized controlled trial. Obstet Gynecol 2001;98:771-778
67. Edelman AB, Gallo MF, Jensen JT, Nichols MD, Schulz KF, Grimes DA. Continuous or extended cycle vs. cyclic use of combined oral contraceptives for contraception. Cochrane Database Syst Rev 2005:CD004695
68. Lethagen S, Harris AS, Sjorin E, Nilsson IM. Intranasal and intravenous administration of desmopressin: effect on F VIII/vWF, pharmacokinetics and reproducibility. Thromb Haemost 1987;58:1033-1036
69. Mannucci PM, Aberg M, Nilsson IM, Robertson B. Mechanism of plasminogen activator and factor VIII increase after vasoactive drugs. Br J Haematol 1975;30:81-93
70. Edlund M, Blombäck M, He S. On the correlation between local fibrinolytic activity in menstrual fluid and total blood loss during menstruation and effects of desmopressin. Blood Coagul Fibrinolysis 2003;14:593-598
71. Rodeghiero F, Castaman G, Mannucci PM. Prospective multicenter study on subcutaneous concentrated desmopressin for home treatment of patients with von Willebrand disease and mild or moderate hemophilia A. Thromb Haemost 1996;76:692-696
72. Leissinger C, Becton D, Cornell CJr, Cox GJ. High-dose DDAVP intranasal spray (Stimate) for the prevention and treatment of bleeding in patients with mild haemophilia A, mild or moderate type 1 von Willebrand disease and symptomatic carriers of haemophilia A. Haemophilia 2001;7:258-266
73. Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. DDAVP nasal spray for treatment of menorrhagia in women with inherited bleeding disorders: a randomized placebo-controlled crossover study. Haemophilia 2002;8:787-793
74. Edlund M, Blombäck M, Fried G. Desmopressin in the treatment of menorrhagia in women with no common coagulation factor deficiency but with prolonged bleeding time. Blood Coagul Fibrinolysis 2002;13:225-231
75. Haynes PJ, Hodgson H, Anderson AB, Turnbull AC. Measurement of menstrual blood loss in patients complaining of menorrhagia. Br J Obstet Gynaecol 1977;84:763-768
76. Coulter A, Kelland J, Peto V, Rees MC. Treating menorrhagia in primary care. An overview of drug trials and a survey of prescribing practice. Int J Technol Assess Health Care 1995;11:456-471
77. Lethaby A, Irvine G, Cameron I. Cyclical progestogens for heavy menstrual bleeding. Cochrane Database Syst Rev 2000:CD001016
78. Irvine GA, Campbell-Brown MB, Lumsden MA, Heikkila A, Walker JJ, Cameron IT. Randomised comparative trial of the levonorgestrel intrauterine system and norethisterone for treatment of idiopathic menorrhagia. Br J Obstet Gynaecol 1998;105:592-598
79. Lethaby A, Augood C, Duckitt K. Nonsteroidal antiinflammatory drugs for heavy menstrual bleeding. Cochrane Database Syst Rev 2000:CD000400
80. Silverberg SG, Haukkamaa M, Arko H, Nilsson CG, Luukkainen T. Endometrial morphology during long-term use of levonorgestrel-releasing intrauterine devices. Int J Gynecol Pathol 1986;5:235-241
81. Stewart A, Cummins C, Gold L, Jordan R, Phillips W. The effectiveness of the levonorgestrel-releasing intrauterine system in menorrhagia: a systematic review. BJOG 2001;108:74-86
82. Lahteenmaki P, Haukkamaa M, Puolakka J, et al. Open randomised study of use of levonorgestrel releasing intrauterine system as alternative to hysterectomy. BMJ 1998;316:1122-1126
83. Hidalgo M, Bahamondes L, Perrotti M, Diaz J, Dantas-Monteiro C, Petta C. Bleeding patterns and clinical performance of the levonorgestrel-releasing intrauterine system (Mirena) up to two years. Contraception 2002;65:129-132
84. Andersson K, Odlind V, Rybo G. Levonorgestrel-releasing and copper-releasing (Nova T) IUDs during five years of use: a randomized comparative trial. Contraception 1994;49:56-72
85. Sivin I, el Mahgoub S, McCarthy T, et al. Long-term contraception with the levonorgestrel 20 mcg/day (LNg 20) and the copper T 380Ag intrauterine devices: a five-year randomized study. Contraception 1990;42:361-378
86. Xiao B, Wu SC, Chong J, Zeng T, Han LH, Luukkainen T. Therapeutic effects of the levonorgestrel-releasing intrauterine system in the treatment of idiopathic menorrhagia. Fertil Steril 2003;79:963-969
87. Kingman CE, Kadir RA, Lee CA, Economides DL. The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disorders. BJOG 2004;111:1425-1428
88. Schaedel ZE, Dolan G, Powell M. The use of the levonorgestrel-releasing intrauterine system in the management of menorrhagia in women with hemostatic disorders. Am J Obstet Gynecol 2005;193:1361-1363
89. Lethaby A, Shepperd S, Cooke I, Farquhar C. Endometrial resection and ablation versus hysterectomy for heavy menstrual bleeding. Cochrane Database Syst Rev 2000: CD000329
90. Lethaby A, Hickey M. Endometrial destruction techniques for heavy menstrual bleeding: a Cochrane review. Hum Reprod 2002;17:2795-2806
91. Maresh MJ, Metcalfe MA, McPherson K, et al. The VALUE national hysterectomy study: description of the patients and their surgery. BJOG 2002;109:302-312
92. McPherson K, Metcalfe MA, Herbert A, et al. Severe complications of hysterectomy, the VALUE study. BJOG 2004;111:688-694