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中重度血管性血友病女性月经过多干预措施及疗效评价。

Review of interventions and effectiveness for heavy menstrual bleeding in women with moderate and severe von Willebrand disease.

机构信息

Katharine Dormandy Haemophilia and Thrombosis Centre, London, UK.

Department of Obstetrics and Gynaecology, Royal Free Hospital NHS Trust, London, UK.

出版信息

Haemophilia. 2024 Sep;30(5):1177-1184. doi: 10.1111/hae.15078. Epub 2024 Jul 21.

Abstract

INTRODUCTION

Women with VWD have an increased risk of gynaecological complications due to haemostatic challenges of menstruation.

AIM

Review gynecological bleeding symptoms and their management in women with moderate-severe VWD.

MATERIALS AND METHODS

Retrospective cohort analysis of prospectively collected data for women with moderate and severe VWD attending a joint multidisciplinary clinic between January 2010 and December 2020. Data was collected from electronic patient records on response to treatment options using PBAC, quality of life (QoL) assessment using SF-36 scores, haemoglobin and ferritin in comparison to pre-treatment values.

RESULTS

Of the 67 women managed in the clinic; all reported heavy menstrual bleeding (HMB). Combination therapy with concurrent hormonal agents and tranexamic acid was required in 80% of women. There was an overall 64% improvement in PBAC scores in the first year, reflecting on QoL with 35% improvement in SF-36 score and correction of anaemia in 21% of cases. The cumulative effect of continued treatment culminated in greater reduction of blood loss, with an overall 71% improvement in PBAC scores by 5 years. One in 10 women required surgical treatment for a gynaecological pathology. Non-compliance was the cause of excessive unscheduled bleeding in 50% of adolescents. After 3 years, one in five women experienced a relapse of symptom, of whom 46% became perimenopausal and 54% discontinued hormonal treatments due to concerns about fertility, hair loss and weight gain.

CONCLUSION

Management of HMB requires careful monitoring and follow-up by MDT with close collaboration between the gynaecology team and HTC. Control of HMB often requires a combination therapy.

摘要

简介

由于月经期间止血方面的挑战,患有血管性血友病(VWD)的女性发生妇科并发症的风险增加。

目的

综述中重度 VWD 女性的妇科出血症状及其管理。

材料和方法

对 2010 年 1 月至 2020 年 12 月期间在联合多学科诊所就诊的中重度 VWD 女性进行前瞻性收集数据的回顾性队列分析。从电子病历中收集有关使用 PBAC 治疗方案的反应、SF-36 评分的生活质量(QoL)评估、血红蛋白和铁蛋白与治疗前值的比较等数据。

结果

在诊所管理的 67 名女性中;所有患者均报告有月经过多(HMB)。80%的女性需要联合激素药物和氨甲环酸进行治疗。第一年 PBAC 评分总体提高了 64%,反映了 QoL 方面的改善,SF-36 评分提高了 35%,21%的病例纠正了贫血。持续治疗的累积效应导致出血量进一步减少,5 年内 PBAC 评分总体提高了 71%。十分之一的女性因妇科疾病需要手术治疗。50%的青少年因不遵医嘱而出现过度非计划性出血。3 年后,五分之一的女性症状复发,其中 46%的女性进入围绝经期,54%的女性因担心生育、脱发和体重增加而停止激素治疗。

结论

HMB 的管理需要 MDT 进行仔细监测和随访,妇科团队和 HTC 之间需要密切协作。控制 HMB 通常需要联合治疗。

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