Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases
Introduction. Bernard-Soulier syndrome (BSS) is a rare inherited bleeding disorder characterized by giant platelets thrombocytopenia e prolonged bleeding timee frequent hemorrhages with considerable morbidity. Data on the outcome of pregnancy and gynecological intervention in BSS are rare a...
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| Format: | Article |
| Language: | English |
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Serbian Medical Society
2014-01-01
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| Series: | Srpski Arhiv za Celokupno Lekarstvo |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0370-8179/2014/0370-81791406351M.pdf |
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| author | Mitrović Mirjana Elezović Ivo Miljić Predrag Antić Darko |
| author_facet | Mitrović Mirjana Elezović Ivo Miljić Predrag Antić Darko |
| author_sort | Mitrović Mirjana |
| collection | DOAJ |
| description | Introduction. Bernard-Soulier syndrome (BSS) is a rare inherited bleeding
disorder characterized by giant platelets thrombocytopenia e prolonged
bleeding timee frequent hemorrhages with considerable morbidity. Data on the
outcome of pregnancy and gynecological intervention in BSS are rare and there
are no general therapeutic recommendations. Cases Outline. We report two
cases of BSS. In the first case a 29-year-old patient with BSS was admitted
in 8 weeks of gestation. The diagnosis of BSS was made on the basis of
prolonged bleeding time, giant-platelets thrombocytopenia, and absent
ristocetin-induced platelet aggregation. In 38 week of gestation Cesarean
section, with platelets transfusion preparation, was performed. Obstetric
intervention passed without complication. Postoperative course was
complicated with a three-week vaginal bleeding resistant to platelet
transfusion. Neonate platelet count was normal. Our second case was a
28-year-old patient with BSS, hospitalized for ovarial tumor surgery. The
patient was prepared for the intervention with platelets transfusion. The
surgery was uncomplicated, but on the second postoperative day a massive
vaginal bleeding, resistant to the platelet transfusion, developed. Bleeding
control was achieved with activated recombinant factor VII. Twelve hours the
patient developed later hypertensive crisis with epileptic seizure due to
subarachnoid hemorrhage. Therapy was continued with platelet transfusion,
antihypertensive and antiedema drugs. PH examination of tumor tissue showed
hemorrhagic ovarial cyst. Conclusion. Obstretic and gynecological
intervention in women with BSS may be associated with a life-threatening
bleeding thus requiring a multidisciplinary approach with adequate
preparation. Because of the limited data in the literature, it is not
possible to provide firm management recommendations and each case should be
managed individually. |
| first_indexed | 2024-12-14T00:55:47Z |
| format | Article |
| id | doaj.art-0f3c4683cd33416283f69a89fd7dc2d0 |
| institution | Directory Open Access Journal |
| issn | 0370-8179 |
| language | English |
| last_indexed | 2024-12-14T00:55:47Z |
| publishDate | 2014-01-01 |
| publisher | Serbian Medical Society |
| record_format | Article |
| series | Srpski Arhiv za Celokupno Lekarstvo |
| spelling | doaj.art-0f3c4683cd33416283f69a89fd7dc2d02022-12-21T23:23:33ZengSerbian Medical SocietySrpski Arhiv za Celokupno Lekarstvo0370-81792014-01-011425-635135510.2298/SARH1406351M0370-81791406351MObstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two casesMitrović Mirjana0Elezović Ivo1Miljić Predrag2Antić Darko3Clinical Center of Serbia, Clinic of Hematology, BelgradeClinical Center of Serbia, Clinic of Hematology, Belgrade + School of Medicine, BelgradeClinical Center of Serbia, Clinic of Hematology, Belgrade + School of Medicine, BelgradeClinical Center of Serbia, Clinic of Hematology, Belgrade + School of Medicine, BelgradeIntroduction. Bernard-Soulier syndrome (BSS) is a rare inherited bleeding disorder characterized by giant platelets thrombocytopenia e prolonged bleeding timee frequent hemorrhages with considerable morbidity. Data on the outcome of pregnancy and gynecological intervention in BSS are rare and there are no general therapeutic recommendations. Cases Outline. We report two cases of BSS. In the first case a 29-year-old patient with BSS was admitted in 8 weeks of gestation. The diagnosis of BSS was made on the basis of prolonged bleeding time, giant-platelets thrombocytopenia, and absent ristocetin-induced platelet aggregation. In 38 week of gestation Cesarean section, with platelets transfusion preparation, was performed. Obstetric intervention passed without complication. Postoperative course was complicated with a three-week vaginal bleeding resistant to platelet transfusion. Neonate platelet count was normal. Our second case was a 28-year-old patient with BSS, hospitalized for ovarial tumor surgery. The patient was prepared for the intervention with platelets transfusion. The surgery was uncomplicated, but on the second postoperative day a massive vaginal bleeding, resistant to the platelet transfusion, developed. Bleeding control was achieved with activated recombinant factor VII. Twelve hours the patient developed later hypertensive crisis with epileptic seizure due to subarachnoid hemorrhage. Therapy was continued with platelet transfusion, antihypertensive and antiedema drugs. PH examination of tumor tissue showed hemorrhagic ovarial cyst. Conclusion. Obstretic and gynecological intervention in women with BSS may be associated with a life-threatening bleeding thus requiring a multidisciplinary approach with adequate preparation. Because of the limited data in the literature, it is not possible to provide firm management recommendations and each case should be managed individually.http://www.doiserbia.nb.rs/img/doi/0370-8179/2014/0370-81791406351M.pdfBernard-Soulier syndromehemorrhagegynecologyobstetrics |
| spellingShingle | Mitrović Mirjana Elezović Ivo Miljić Predrag Antić Darko Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases Srpski Arhiv za Celokupno Lekarstvo Bernard-Soulier syndrome hemorrhage gynecology obstetrics |
| title | Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases |
| title_full | Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases |
| title_fullStr | Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases |
| title_full_unstemmed | Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases |
| title_short | Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: Report of two cases |
| title_sort | obstetric and gynecological intervention in women with bernard soulier syndrome report of two cases |
| topic | Bernard-Soulier syndrome hemorrhage gynecology obstetrics |
| url | http://www.doiserbia.nb.rs/img/doi/0370-8179/2014/0370-81791406351M.pdf |
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