Department of Pediatrics, Division of Hematology/Oncology, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Department of Pediatrics, Division of Hematology/Oncology, Emory University, Atlanta, Georgia, USA.
Haemophilia. 2022 May;28 Suppl 4(Suppl 4):18-25. doi: 10.1111/hae.14535.
Women and girls affected by haemophilia, including haemophilia carriers (WGH) are at risk of bleeding symptoms that may go unrecognized, including heavy menstrual bleeding (HMB) and musculoskeletal bleeding. Terminology continues to evolve.
To describe the current recommendations for nomenclature surrounding WGH, and the current understanding of HMB, iron deficiency, and musculoskeletal complaints in these patients.
Literature was reviewed and summarized.
With regards to nomenclature, women with factor levels less than 50% should be classified as having haemophilia, while carriers with normal levels should be characterized accordingly to symptomatology. HMB and resultant iron deficiency are common among WGH, have a multitude of downstream effects, and maybe overlooked due to stigma around menstruation. Musculoskeletal bleeding and resultant joint changes are increasingly recognized in this population but do not necessarily correlate with factor levels.
Although progress has been made in the care of WGH, much work remains to further improve their care.
受血友病影响的女性和女孩，包括血友病携带者（WGH），有出血症状的风险，这些症状可能未被识别，包括月经过多（HMB）和肌肉骨骼出血。术语仍在不断发展。
描述目前围绕 WGH 的命名建议，以及目前对这些患者的 HMB、缺铁和肌肉骨骼投诉的理解。
对文献进行了回顾和总结。
关于命名法，因子水平低于 50%的女性应被归类为患有血友病，而正常水平的携带者应根据症状进行相应的特征描述。WGH 中常见 HMB 和由此导致的缺铁，有多种下游影响，由于月经的耻辱感，可能会被忽视。肌肉骨骼出血和由此产生的关节变化在这一人群中越来越被认识到，但不一定与因子水平相关。
尽管在 WGH 的护理方面已经取得了进展，但仍有许多工作要做，以进一步改善他们的护理。
