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Gynaecological Problems in Severe Congenital Factor VII DeficiencyFrom: Fenn (fennmoses@gmail.com)Thu Mar 6 02:21:31 2008
Congenital F VII deficiency is the most common of the ‘rare bleeding disorders’ with a prevalence of severe cases of 1:100 000 in Slovakia. The higher proportion of females in our series of 54 severely affected patients (F/M:33/21) and significantly higher frequency of serious bleeding in females than in males during adolescence and adult age, point on the dominant gynaecological problems in females with FVII level <2%. We have reviewed the clinical course of the fertile age of 24 severe FVII deficient females (M 44 yrs, range 16–70 years), with median FVII:C/FVII:Ag levels of 0.9%/1.0%, respectively. Out of 20 females genetically tested 13, 4 and 3 were homozygous for Ala294Val, 404 delC, double heterozygous Ala294Val and -62 CT, Cys310Phe/ Glu35Glu mutation, respectively. Serious menstrual bleeding at the start of menarché and later menorrhagia during the fertile age occurred in 17 (72%) and 23 (96%) patients, respectively. Seventeen of 23 (74%) patients with menorrhagia required RBC (in six patients occurred an acute decrease of hemoglobin to 35–50 g/L), 14 (61%) underwent therapeutic curettage and 16 (69%) have been receiving irregular hormonal therapy, respectively. Ovarial cysts and intraabdominal ovulation bleeding developed in 15/24 (63%) and 4 (17%) patients, respectively, with an urgent surgery in three. Ovarectomy was done in 10 (42%) and hysterectomy in 11 (46%) patients at a median age of 29 years and 35 years, respectively. Fourteen patients had in total 36 pregnancies without bleeding or gestational complications. Serious postpartum bleeding occurred in 3 of 4 females not receiving prophylactic therapy, while the bleeding complications were prevented in all 10 patients treated with factor replacement before delivery. Conclusion: Serious gynaecological problems are common in severe FVII deficient woman. The previous need of early gynecological surgery currently in most cases may be prevented by an adequate factor replacement and systematic hormonal therapy. source: http://www.blackwellpublishing.com/isth2005/abstract.asp?id=46287#top
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