Pregnancy and Delivery in Bernard‐Soulier Syndrome

Michalas, Stylianos; Malamitsi-Puchner, A; Tsevrenis, H

doi:10.3109/00016348409154659

Cited by 21 publications

(31 citation statements)

References 5 publications

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“…In all twenty of these articles, the method of blood loss estimation (eg weighted or visual estimation) was not specified. Other studies defined PPH as blood loss ≥ 500 mL without further specifying the exact amount of blood loss or elaboration on the definition of PPH . Overall, PPH occurred in 63.2% (55/87) of deliveries.…”

Section: Resultsmentioning

confidence: 99%

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Menstrual and obstetrical bleeding in women with inherited platelet receptor defects—A systematic review

et al. 2020

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Introduction Women with inherited platelet receptor defects (IPRD) may have an increased risk of heavy menstrual bleeding (HMB) and postpartum haemorrhage (PPH). Aim To present a systematic overview of the literature on the prevalence and management of menstrual and obstetrical bleeding in women with IPRD. Methods Electronic databases were searched for original patient data on the prevalence and management of HMB and PPH in women with known IPRD or who were being investigated for IPRD. Results Sixty‐nine papers (61 case reports/series and 8 cohort studies) were included. Overall, studies were rated as ‘poor quality’. The included cohort studies reported HMB in 25% (13/52) of women with Bernard‐Soulier syndrome and in 22.1% (34/154) of women with Glanzmann thrombasthenia. In total, 164 deliveries in women with IPRD were described. Excessive bleeding occurred in 16.9% (11/65) of deliveries described in the largest cohort. PPH occurred in 63.2% (55/87) of deliveries described in case reports/series. PPH occurred in 73.7% (14/19) of deliveries that were not covered by prophylaxis compared with 54.2% (32/59) of deliveries that were (OR = 2.36, 95% CI 0.75‐7.40). Neonatal bleeding complications were reported in 10.0% (8/80) of deliveries. In all (6/6) deliveries with neonatal bleeding complications wherein the presence of alloantibodies was investigated, either antiplatelet or anti‐HLA antibodies were detected. Discussion/Conclusion Menstrual and particularly obstetrical bleeding problems frequently occur in women with IPRD, based on small case reports and series of poor quality. International collaboration, preferably on prospective studies, is needed to improve clinical management of women‐specific bleeding in IPRD.

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Section: Resultsmentioning

confidence: 99%

“…References: small studies, 20-37, 39-61, 63-77, 79 medium studies, 13,38,62,78,83,86 large studies. 4 women.…”

Section: Data From Case Reports and Seriesmentioning

confidence: 99%

Menstrual and obstetrical bleeding in women with inherited platelet receptor defects—A systematic review

et al. 2020

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“…2 In some patients, the pregnancy course is smooth, whereas in others postpartum hemorrhage is the most common complication. [5][6][7] Transfusions of allogeneic platelets are the only treatment with proven effect for patients with Bernard-Soulier syndrome. 2,5 Although each unit of platelet concentrate is expected to raise the platelet count of an adult without platelet disorders by approximately 5,000 to 10,000/L, the effect of platelet transfusion on platelet count in patients with Bernard-Soulier syndrome has never been quantified.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic and perioperative management of a patient with Bernard-Soulier syndrome

Kostopanagiotou

Siafaka

Sikiotis

et al. 2004

Journal of Clinical Anesthesia

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“…, 1999). Uncontrolled postpartum haemorrhage necessitated a hysterectomy in one of the other patients (Michalas, Malamitsi‐Puchner & Tsevrenis, 1984).…”

Section: Discussionmentioning

confidence: 99%

“…The clinical course is variable between different patients and between different pregnancies in the same woman. There have been nine case reports on pregnant women with BSS (Michalas, Malamitsi‐Puchner & Tsevrenis, 1984; Heslop et al. , 1986; Peaceman, Katz & Laville, 1989; Peng et al.…”

Section: Introductionmentioning

confidence: 99%

Bernard-Soulier syndrome in pregnancy

Prabu

Parapia

2006

Clin Lab Haematol

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Bernard-Soulier syndrome (BSS) is a rare autosomal recessively inherited bleeding disorder. Pregnancy in patients with BSS is characterized by ante-, intra-, or postpartum haemorrhage, which may be delayed and severe. There is no consensus in the management of BSS in pregnancy and so far only 16 pregnancies in nine patients have been described. We report a further three pregnancies in two women with the syndrome. We also outline our management of pregnant patients with BSS.

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Pregnancy and Delivery in Bernard‐Soulier Syndrome

Cited by 21 publications

References 5 publications

Menstrual and obstetrical bleeding in women with inherited platelet receptor defects—A systematic review

Menstrual and obstetrical bleeding in women with inherited platelet receptor defects—A systematic review

Anesthetic and perioperative management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome in pregnancy

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