Von Willebrand’s Disease (vWD) Among women with unexplained menorrhagia.

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Presentation transcript:

Von Willebrand’s Disease (vWD) Among women with unexplained menorrhagia

vWD Prevalence The commonest congenital bleeding disorder at 1 to 2/100 Autosomal inheritance, mostly dominant Multiple different types comprising quantitative or qualitative defects of von Willebrand Factor (vWF) Considerable heterogeneity in clinical phenotype and in genotype

vWD Clinical Perspective 1 Commonest signs: epistaxes; menorrhagia; post-operative bleeds; oral and GI bleeding (angiodysplasia) Both genders affected equally, but menses, pregnancy & childbirth mean women are more vulnerable. Over 60% of vWD patients are women (Cabrera, 1989) Menorrhagia (>80 ml/cycle) and iron deficiency in >65%. Begins at menarche.

vWD Clinically 2 Morbidity in a critical sector of society, when demands of family and work are greatest Lack of awareness of medical community No objective criteria for estimating blood loss Cultural barriers exist in Iran, preventing discussion. In affected families, menorrhagia may be considered normal Pregnancy usually well tolerated

vWD Clinically 3 Prevalence of vWD in women with menorrhagia is greater than perceived: 11 studies of 998 women with menorrhagia showed 13% had some form of vWD (Kadir, 2004) Average time to diagnosis in US: 16 years due to unawareness. In UK & US, only 2% and 4% of specialists considered vWD screening for menorrhagia Unawareness may lead to early hysterectomy

vWD Clinically 4 US College of Obst/Gynae (2001) recommended vWD screening for: 1.Adolescents with severe menorrhagia 2.Women before hysterectomy for menorrhagia 3.Adults where no cause for menorrhagia found Kirtava, 2003, reports other gynae. problems in vWD: dysmenorrhea (in 50%); ovarian cyst; endometriosis; even haemoperitoneum

Diagnosis of vWD Laboratory investigation complex and not widely available: ABO Blood Group; Template Bleeding Time; PFA- 100 closure time; Platelet count; FVIII:C; vWF:Ag; vWF: RiCoF; vWF:CB; vWF: FVIIIB; RIPA; HMW Multimer Analysis; Molecular Genetic studies; MCMDM-1vWD Questionnaire (70% sensitive; 98% specific)