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先天性无纤维蛋白原血症女性复发性大量腹腔积血与黄体破裂相关;病例报告

Recurrent massive haemoperitoneum associated with ruptured corpus luteum in women with congenital afibrinogenemia; case report.

作者信息

Özdemir Özhan, Sarı Mustafa Erkan, Kurt Aslıhan, Şen Ertuğrul, Atalay Cemal Reşat

机构信息

Ankara Numune Education and Research Hospital, Clinic of Gynecology and Obstetrics, Ankara, Turkey.

出版信息

Turk J Obstet Gynecol. 2014 Dec;11(4):242-245. doi: 10.4274/tjod.04935. Epub 2014 Dec 15.

Abstract

Massive hemoperitoneum secondary to ruptured corpus luteum is a rare but serious and life-threatening complication for women with congenital bleeding disorders and may lead to surgical interventions and even oophorectomy. Congenital afibrinogenemia is a rare inherited coagulation disorder. As it can be asymptomatic, its clinical manifestations vary from minimal tendency of bleeding to life-threatening bleedings. Intraabdominal bleeding due to ovulation is very rare in these patients and only a few cases of corpus luteum rupture and hemoperitoneum in afibrinogenemic patients have been described. We report on a 28-year-old woman with congenital afibrinogenemia with recurrent massive intraabdominal bleeding due to ovulation as the presenting clinical sign. The first episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions; exploratory laparotomy and excision of the ruptured follicle was performed at the second episode; the third episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions; exploratory laparotomy and right salphingooopherectomy was performed at the fourth episode; fifth episode was managed with fresh frozen plasma, cryoprecipitate and blood transfusions. Conservative management is crucial for patients with congenital bleeding disorders. These case demonstrate that preservation of ovarian function is possible with a conservative approach and recurrent episodes may be prevented by suppression of ovulation.

摘要

黄体破裂继发的大量腹腔积血对于患有先天性出血性疾病的女性来说是一种罕见但严重且危及生命的并发症,可能导致手术干预甚至卵巢切除术。先天性无纤维蛋白原血症是一种罕见的遗传性凝血障碍。由于其可能无症状,其临床表现从轻微出血倾向到危及生命的出血不等。这些患者因排卵导致的腹腔内出血非常罕见,仅有少数关于无纤维蛋白原血症患者黄体破裂和腹腔积血的病例报道。我们报告一例28岁先天性无纤维蛋白原血症女性患者,以排卵导致反复大量腹腔内出血为主要临床症状。首次发作时采用新鲜冰冻血浆、冷沉淀和输血治疗;第二次发作时进行了剖腹探查和破裂卵泡切除术;第三次发作时采用新鲜冰冻血浆、冷沉淀和输血治疗;第四次发作时进行了剖腹探查和右侧输卵管卵巢切除术;第五次发作时采用新鲜冰冻血浆、冷沉淀和输血治疗。保守治疗对于先天性出血性疾病患者至关重要。这些病例表明,采用保守方法有可能保留卵巢功能,并且通过抑制排卵可以预防复发。

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