Bernard Soulier syndrome in pregnancy: a systematic review.

Bernard Soulier syndrome (BSS) is a rare disorder of platelets, inherited mainly as an autosomal recessive trait. It is characterised by qualitative and quantitative defects of the platelet membrane glycoprotein (GP) Ib-IX-V complex. The main clinical characteristics are thrombocytopenia, prolonged bleeding time and the presence of giant platelets. Data on the clinical course and outcome of pregnancy in women with Bernard Soulier syndrome is scattered in individual case reports. In this paper, we performed a systematic review of literature and identified 16 relevant articles; all case reports that included 30 pregnancies among 18 women. Primary postpartum haemorrhage was reported in 10 (33%) and secondary in 12 (40%) of pregnancies, requiring blood transfusion in 15 pregnancies. Two women had an emergency obstetric hysterectomy. Alloimmune thrombocytopenia was reported in 6 neonates, with one intrauterine death and one neonatal death. Bernard Soulier syndrome in pregnancy is associated with a high risk of serious bleeding for the mother and the neonate. A multidisciplinary team approach and individualised management plan for such women are required to minimise these risks. An international registry is recommended to obtain further knowledge in managing women with this rare disorder.

Management of a pregnancy complicated by yolk sac tumor.

BACKGROUND: The management of ovarian cancer during pregnancy represents a major challenge and requires close multidisciplinary team approach. CASE: A 35-year-old pregnant woman with a yolk sac tumor underwent left salpingo-oophorectomy at 25 weeks of gestation. Chemotherapy was deferred to the end of the pregnancy owing to concerns for potential fetal risks. Alpha-feto protein level was used to monitor the underlying disease activity. The patient underwent exploratory laparotomy with cesarean section followed by total hysterectomy, omentectomy, right salpingooophorectomy, pelvic, and para-aortic lymphadenectomies at 32 weeks of gestation. She received four postoperative courses of chemotherapy (cisplatin, etoposide, and peplomycin). Currently, mother and child are doing well 6 months after the last chemotherapy cycle. CONCLUSION: In a case of yolk sac tumor in the second trimester of pregnancy, radical surgery combined with elective caesarian section followed by chemotherapy could achieve remission and rescue of fetus. However, the treatment needs to be individualized as there is lack of evidence.

Cervical leukocyte sub-populations in idiopathic preterm labour.

OBJECTIVE: To investigate cervical epithelial leukocyte sub-populations in pregnant women with a history of idiopathic preterm labour. METHODS: A prospective observational study was undertaken of 106 women with a past history of idiopathic preterm delivery following spontaneous labour. A cytobrush was used to sample the epithelium of the cervix at 12-16 weeks of gestation and again 8 weeks later. All women had investigations for cervical and vaginal infection as well as serial transvaginal ultrasonography of their cervix; the mode and gestation at delivery were recorded. Leukocyte sub-populations were examined using immunocytochemistry, and the number of leukocytes per total cell count was calculated. MAIN OUTCOME MEASURES: Cervical epithelial leukocytes populations were (1) described in pregnancy, (2) observed over increasing gestation, (3) analysed in women who developed marked cervical shortening and (4) in those whose preterm labour recurred. RESULTS: There was no significant change in cervical epithelial leukocyte populations during the second trimester of pregnancy. There was no association between cervical leukocytes and cervical shortening. Women with idiopathic preterm labour that recurred had fewer cervical macrophages at the beginning of the second trimester of pregnancy than those whose subsequent pregnancy progressed beyond 35 weeks of gestation. CONCLUSIONS: Cervical epithelial macrophages may serve to prevent recurrent preterm labour, possibly by preventing ascending infection.