Bleeding assessment in haemophilia carriers—High rates of bleeding after surgical abortion and intrauterine device placement: A multicentre study in China

Li, Sen; Fang, Yunhai; Li, Li; Lee, Adrienne; Poon, Man‐Chiu; Zhao, Yongqiang; Zhang, Xinsheng; Wang, Shujie; Cheng, Yan; Li, Kuixing

doi:10.1111/hae.13889

Cited by 10 publications

(15 citation statements)

References 13 publications

(53 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There were also no significant differences in factor levels between carriers with high (≥4) and low (<4) BAT scores. In the Chinese cohort, investigators did not find any significant correlation between ISTH‐BAT scores and factor levels 16 . All of these cohorts did provide evidence that even carriers with normal baseline FVIII or FIX levels can have abnormal BAT scores, and at an increased frequency compared to the general population.…”

Section: Bleeding In Women and Girls With Haemophilia – Epidemiology Anmentioning

confidence: 90%

“…Studies from Belgium, Sweden and the United States have shown that cutaneous bleeding, epistaxis and postsurgical bleeding are also commonly reported by WGWH 13‐15 . In a cohort of 145 carriers in China (accompanying male relatives rather than referred for clinical management themselves), participants completed a modified ISTH‐BAT score, which included additional questions regarding bleeding after intrauterine device placement and surgical abortion 16 . Carriers had higher BAT scores than controls (3 vs 1, P <.001), and also reported high rates of excessive bleeding after intrauterine device placement (36%, 21/58) and surgical abortion (41%, 33/80).…”

Section: Bleeding In Women and Girls With Haemophilia – Epidemiology Anmentioning

confidence: 99%

See 1 more Smart Citation

Women and girls with haemophilia: Lessons learned

2020

View full text Add to dashboard Cite

Severe and moderate factor VIII (FVIII) or IX (FIX) deficiencies in female carriers of haemophilia are rarely observed, but mild deficiency is quite frequent, although insufficiently recognized and registered. The confusion between the genetic diagnosis of the carriership, mainly assessed at adult age and the diagnosis of the bleeding disorder for those who have low factor levels often prevents early diagnosis of a potential bleeding risk. The factor levels in obligate or potential carriers of haemophilia can be assessed during childhood, possibly apart from genetic assays. The absence of early recognition of the bleeding disorder precludes the anticipation of menarche and the prevention of potential heavy menstrual bleeding to heavy menstrual bleeding. Standardized bleeding assessment tools (BAT) have demonstrated that women and girls with haemophilia (WGWH) have increased bleeding scores as compared to the general female population, however weakly correlating with factor levels. More recent evidence has highlighted that hemarthroses affect 4% to 19% of carriers and that some of them could experience sub‐clinical joint bleeding. Desmopressin for women with FVIII deficiency and abnormal ISTH‐BAT scores had a significantly lower FVIII response to DDAVP compared to those with normal bleeding scores, which could at least partially explain more postsurgical bleeding. Management of delivery of haemophilia carriers requires attention to the risks of maternal bleeding, the risks of foetal bleeding, preconception and prenatal care, strategies to reduce maternal bleeding, choice of mode of delivery to reduce foetal/neonatal bleeding, and postpartum care. Either prior to pregnancy, or during early pregnancy, a plan should be developed that addresses the needs of both the mother and her unborn baby. If the unborn baby is a male proven to be or potentially affected by moderate or severe form of haemophilia, there is a risk of severe foetal bleeding, so a planned caesarean delivery may be preferred. If the unborn baby is a carrier, or potentially affected carrier, there is still the risk of non‐severe bleeding so invasive foetal procedures and operative vaginal delivery (forceps or vacuum) should be avoided. Further studies based on large cohorts will help the community to favour earlier diagnosis, increase knowledge on WGWH and promote better care.

show abstract

Section: Bleeding In Women and Girls With Haemophilia – Epidemiology Anmentioning

confidence: 90%

Section: Bleeding In Women and Girls With Haemophilia – Epidemiology Anmentioning

confidence: 99%

Women and girls with haemophilia: Lessons learned

2020

View full text Add to dashboard Cite

show abstract

“…This should provide a more robust data set going forward. Finally, our analysis portrays a subset of haemophilia carriers residing in the United States and may not be applicable to other areas of the world 13,23 …”

Section: Discussionmentioning

confidence: 99%

Descriptive analysis of bleeding symptoms in haemophilia carriers enrolled in the ATHNdataset

Puetz

Cheng

2021

Haemophilia

View full text Add to dashboard Cite

Background: Several studies have reported that haemophilia carriers have a bleeding tendency independent of factor activity. However, investigations have been fraught with methodological concerns. The ATHNdataset houses the largest data set of haemophilia carriers in the world. We undertook an analysis of haemophilia carriers in this data set using methodologies that characterize bleeding symptoms in carriers.Aim: Determine the proportion of haemophilia carriers who have a normal bleeding score (BLS) and factors that affect the BLS. Methods:The ATHNdataset was queried for haemophilia carriers with a documented BLS. Collected data included demographics, ISTH-BAT score, factor activity level, type of haemophilia (A or B), genotype and geographic residence.Results: Nine hundred twenty-two haemophilia carriers in the ATHNdataset reported a BLS. When adjusted for age, 74% reported a normal score. Logistic regression identified age, factor activity level, ethnicity and region of residence as risk factors for an abnormal score. Conclusions:The majority of haemophilia carriers (74%) in the ATHNdataset had a normal BLS, including the majority (59%) with factor activity levels < 40 IU/dl. Conversely, 24% of haemophilia carriers with a factor activity level >40 IU/dl reported an abnormal BLS. These results are consistent with previous studies of haemophilia carriers. Additional investigation is needed to determine why a majority of haemophilia carriers with low factor activity levels report normal BLSs while a significant minority of haemophilia carriers with normal activity levels report abnormal BLSs.

show abstract

“…1,2 The common misconception by patients and health-care providers (HCPs) that all HCs are asymptomatic is shifting, due to an increasing body of evidence that contradicts this assumption. [3][4][5][6][7] The term HC, and its associated connotations, such as obligate and possible carriers, has hampered diagnosis, management, and research. 8,9 For each man with hemophilia, 1.6 female carriers can be identified.…”

Section: Backg Round and R Ationalementioning

confidence: 99%

“…Due to lyonization, a random interindividual X‐linked inactivation process, median plasma factor VIII/IX (FVIII/IX) levels of hemophilia carriers (HCs) are 0.60 IU/ml (range 0.05–2.19), and 28% have factor levels <0.40 IU/ml, which would be defined as hemophilia according to the World Federation of Hemophilia (WFH) 1,2 . The common misconception by patients and health‐care providers (HCPs) that all HCs are asymptomatic is shifting, due to an increasing body of evidence that contradicts this assumption 3–7 . The term HC, and its associated connotations, such as obligate and possible carriers, has hampered diagnosis, management, and research 8,9 …”

Section: Background and Rationalementioning

confidence: 99%

A new hemophilia carrier nomenclature to define hemophilia in women and girls: Communication from the SSC of the ISTH

Galen

d’Oiron

James

et al. 2021

Journal of Thrombosis and Haemostasis

View full text Add to dashboard Cite

Hemophilia A and B predominantly attracts clinical attention in males due to X‐linked inheritance, introducing a bias toward female carriers to be asymptomatic. This common misconception is contradicted by an increasing body of evidence with consistent reporting on an increased bleeding tendency in hemophilia carriers (HCs), including those with normal factor VIII/IX (FVIII/IX) levels. The term HC can hamper diagnosis, clinical care, and research. Therefore, a new nomenclature has been defined based on an open iterative process involving hemophilia experts, patients, and the International Society on Thrombosis and Haemostasis (ISTH) community. The resulting nomenclature accounts for personal bleeding history and baseline plasma FVIII/IX level. It distinguishes five clinically relevant HC categories: women/girls with mild, moderate, or severe hemophilia (FVIII/IX >0.05 and <0.40 IU/ml, 0.01–0.05 IU/ml, and <0.01 IU/ml, respectively), symptomatic and asymptomatic HC (FVIII/IX ≥0.40 IU/ml with and without a bleeding phenotype, respectively). This new nomenclature is aimed at improving diagnosis and management and applying uniform terminologies for clinical research.

show abstract

Bleeding assessment in haemophilia carriers—High rates of bleeding after surgical abortion and intrauterine device placement: A multicentre study in China

Cited by 10 publications

References 13 publications

Women and girls with haemophilia: Lessons learned

Women and girls with haemophilia: Lessons learned

Descriptive analysis of bleeding symptoms in haemophilia carriers enrolled in the ATHNdataset

A new hemophilia carrier nomenclature to define hemophilia in women and girls: Communication from the SSC of the ISTH

Contact Info

Product

Resources

About