Objectives To determine how menorrhagia is managed in people with bleeding disorders.

Design A systematic review and thematic synthesis.

Data sources PubMed, Medline, Scopus, Cochrane library, google scholar and CINAHL complete (via EBSCO).

Methods Searches were conducted on articles published from 1st January 2000 until 6th May 2024. Following deduplication, the titles and abstracts were screened for relevance. 244 primary studies were then assessed for eligibility based on inclusion and exclusion criteria. Studies were included if they were based on primary articles and focussed on people with inherited bleeding disorders and heavy menstrual bleeding. Included studies were appraised for risk of bias and quality assurance using the Newcastle Ottawa Scale, following which data was systematically coded to generate descriptive and analytical themes.

Results We identified 16 eligible articles of which 13 were included in a thematic synthesis. These included prospective and retrospective clinical studies, cross-sectional studies and randomised control trials encompassing over 893 participants. Thematic synthesis identified hormonal treatments, such as the levonorgestrel-releasing intrauterine system (LNG-IUS), to be largely effective in the symptom management of HMB in IBD and associated with improved quality of patient life.

Treatment of HMB patients with LNG-IUS, followed by tranexamic acid (TA) or 1-deamino-8-d-arginine vasopressin (DDAVP), the trade name for desmopressin, commonly led to amenorrhea. Technological approaches to the management of HMB in IBD included the use of mobile technology to encourage treatment compliance. These management strategies led to an improvement in reported QoL by patients with IBD. This review had limitations including the exclusion of some articles that may have limited generalisability. The Medical Subject Heading (MeSH) terms used focussed on HMB as opposed to abnormal menstrual bleeding, potentially directing the identified recommendations for clinical practice. Based on the findings of this thematic review, the use of LNG-IUS as first line therapy for those with HMB, followed by the use of combination therapy such as TA and desmopressin, would be recommended. These measures should be adopted in both primary and secondary care settings. We identified the need to strengthen counselling and communication between specialists involved in the care of those with HMB and IBD, and the need to increase awareness of HMB in IBD through public and patient education.

Data availability statement All data presented is secondary to published studies and available within the public domain.

Registration PROSPERO registration number: CRD42023452533

Key Messages

What is already known on this topic – Heavy menstrual bleeding (HMB) is often a symptom of inherited bleeding disorders (IBD) in females and can have a significant impact on the quality of life of an individual.

What this study adds – A systematic review and thematic analysis of the currently available literature allowed the identification of best practise management options for patients with IBD and HMB. A thematic synthesis was used to identify best practice for IBD patient treatment and management of HMB, which will improve patient quality of life.

How this study might affect research, practice or policy – This study of existing literature and thematic synthesis has been used to provide recommendations to haematologists and gynaecologists to support evidence based best practise recommendations on how to treat patients with HMB consequent to IBDs.

The authors have declared no competing interest.

This work was funded in part through an INSPIRE grant awarded to the Hull York Medical School by the Academy of Medical Sciences through the Wellcome Trust [Ref: IR5\1018].

I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained.

Yes

The details of the IRB/oversight body that provided approval or exemption for the research described are given below:

The study used ONLY openly available human data that were originally located in articles citated in this article.

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Yes

I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance).

Yes

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Yes

Abbreviations: DDAVP: 1-deamino-8-d-arginine vasopressin; EAHAD: The European Association for Haemophilia and Allied Disorder; FIGO: Federation of Gynaecology and Obstetrics; HMB: Heavy Menstrua Bleeding; IBD: Inherited Bleeding Disorder; LNG-IUS: levonorgestrel-releasing intrauterine system; NICE: the National Institute for Health and Care Excellence; NSAID: Non-steroidal anti-inflammatory; PBAC: Pictorial Blood Assessment Chart; QoL: Quality of Life; rVWF: recombinant Von Willebrand Factor; TA: Tranexamic Acid; VWD: Von Willebrand Disease; VWF: Von Willebrand Factor

The data generated and/or analysed during the current study are available as Supplementary Material at DOI: 10.5281/zenodo.15061468.