Hasani Maria, Shriya Sadia Rounak, Thahira Mariyam, Tayb Tasnima, Yaseen Hasan Aal
Internal Medicine, Mohammed Bin Rashid University of Medicine and Health Sciences, Dubai Health, Dubai, ARE.
Hematology, Dubai Hospital, Dubai Health, Dubai, ARE.
Cureus. 2024 Dec 22;16(12):e76233. doi: 10.7759/cureus.76233. eCollection 2024 Dec.
Bernard-Soulier syndrome (BSS) is a rare qualitative condition of platelets wherein deficiency of platelet surface glycoproteins (GP) Ib, IX, and V forms the Ib-IX-V complex, leading to impaired hemostasis. Although it commonly presents as prolonged bleeding in general, women in the reproductive phase report additional complications during menstruation, pregnancy, and childbirth. In women of reproductive age, menorrhagia is a frequent complaint. It is reported that secondary to hormonal and other causes, hemostatic disorders are present in a substantial proportion of women presenting with persistent menorrhagia. Women with bleeding disorders also hold an additional risk of developing complications like hemorrhagic corpus luteum. While corpus luteum rupture may go unnoticed in healthy women, it can cause life-threatening intraperitoneal hemorrhage in women with bleeding disorders. This report presents a case of a young woman with BSS who developed menorrhagia against the backdrop of a hemorrhagic ovarian cyst. Owing to the rarity of reported management of hemorrhagic cysts with underlying BSS, this report aimed to potentially serve as a guide in facilitating decision-making for physicians.
伯纳德-索利尔综合征（BSS）是一种罕见的血小板质性疾病，其中血小板表面糖蛋白（GP）Ib、IX和V缺乏会形成Ib-IX-V复合物，导致止血功能受损。虽然一般来说它通常表现为出血时间延长，但处于生殖期的女性在月经、怀孕和分娩期间会出现额外的并发症。在育龄女性中，月经过多是常见的主诉。据报道，由于激素和其他原因，相当一部分持续性月经过多的女性存在止血障碍。患有出血性疾病的女性还存在额外的风险，如发生出血性黄体等并发症。虽然健康女性的黄体破裂可能未被注意到，但在患有出血性疾病的女性中，它可能会导致危及生命的腹腔内出血。本报告介绍了一例患有BSS的年轻女性病例，该女性在出血性卵巢囊肿的背景下出现了月经过多。由于报道的合并潜在BSS的出血性囊肿治疗案例罕见，本报告旨在可能为医生的决策提供指导。
