Pregnancy in women with von Willebrand's disease or factor XI deficiency

Abstract

Objective To assess the obstetric outcome in women with von Willebrand's disease or factor XI Setting Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital.Population Women with von Willebrand's disease (n = 3 1) and with factor XI deficiency (n = 11) registered at the Royal Free Hospital Haemophilia Centre who had had a pregnancy within the previous 17 years (1980)(1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficiency.Methods Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's maternity records were also reviewed.Results Threatened miscarriage occurred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recwent miscarriages, 14/68 (21 %) of pregnancies with von Willebrand's disease and one pregnancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding complications. Factor VIJI and von Willebrand factor antigen and activity levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did not show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bruising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incidence of primary postpartum haemorrhage was 185% in von Willebrand's disease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI deficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophylactic treatment for labour. The incidence of secondary postpartum haemorrhage was 20% in von Willebrand's disease and 24% in factor XI deficiency. None of the women who had prophylactic treatment during labour or the puerperium suffered any significant bleeding complications. There were three neonatal bleeding complications.Conclusion Pregnancy, labour and the puerperium are associated with significant bleeding problems in women with von Willebrand's disease or factor XI deficiency, but these are largely preventable. SDecialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal deficiency.and neonatal complications.

Pregnancy and delivery in women with von Willebrand's disease and different von Willebrand factor mutations

“…Given the case selection, the large majority of these women had a severe phenotype, with FVIII:C and/or VWF basal levels less than 20 IU/dL (Table I), at variance with women previously reported in other studies. 4 In mild cases of VWD, normalization of FVIII and VWF activities at the end of pregnancy is usually observed and prophylactic treatment is seldom required.…”

“…1,2 While patients with mild deficiency usually show a spontaneous complete correction of basal low factor VIII (FVIII) and von Willebrand factor (VWF) levels during pregnancy, patients with dysfunctional (type 2) or severe (type 3) VWD usually require replacement treatment to avoid immediate or delayed bleeding. 3,4 Despite the fact that several studies have addressed the risk of bleeding at parturition, very few have evaluated FVIII and VWF changes during pregnancy, the bleeding risk and treatment modality in relationship to the specific VWF gene mutations and response to desmopressin. We recently reported a favorable clinical outcome at delivery using desmopressin in a few women with VWD associated with C1130F mutation 5 and R1205H (VWD Vicenza), 6 in whom FVIII and VWF increased poorly during pregnancy.…”

“…The low rate of miscarriage and vaginal bleeding during pregnancy observed in our patients is at variance with previous observations in a series of patients retrospectively evaluated during 1980-1996. 4 The identification of mutations among VWD patients is important but cannot be considered the sole parameter useful for determining the clinical severity of VWD, a disorder with wide heterogeneity of phenotypes. Indeed, in this respect, the bleeding score seems to represent a better instrument for appreciating the real risk of bleeding in specific circumstances.…”

Beyond maternal death: improving the quality of maternal care through national studies of ‘near-miss’ maternal morbidity

“…Several studies have demonstrated individual medical comorbidities, such as pre-existing asthma, hypertension, malignancy, chronic ischaemic and congenital heart disease, chronic renal disease, systemic lupus erythematosus, hypercoagulability states, human immunodeficiency virus and diabetes mellitus, to be associated with both severe maternal morbidity and mortality, 25,153,[162][163][164][165][166][167][168] but the extent of the population risk attributable to medical comorbidities as a whole in the UK has not previously been quantified. Uptake of antenatal care was found to be poorer among women with medical comorbidities in our study population, which could increase the adverse effects associated with these conditions.…”

Building our global family – achieving treatment for all

“…PPH can be prevented or its severity can be moderated by educating women and their providers [8]. While delayed or secondary PPH is rare, occurring after <1% of deliveries [10,11], it has been reported in 20-25% of women with VWD [12,13], 2-11% of haemophilia carriers [14,15] and 24% of women with factor XI deficiency [14]. Carriers are another significant and often neglected member of the global bleeding disorder family.…”