Evaluation of the utility of the <scp>ISTH</scp>‐<scp>BAT</scp> in haemophilia carriers: a multinational study

James, Paula; Mahlangu, Johnny; Bidlingmaier, Christoph; Mingot‐Castellano, María Eva; Chitlur, Meera; Fogarty, Patrick F.; Cuker, Adam; Mancuso, Maria Elisa; Holme, Pål André; Grabell, Julie; Satkunam, Natasha; Hopman, Wilma M.; Mathew, Prasad; Panel, Global Emerging HEmostasis Experts

doi:10.1111/hae.13089

Cited by 72 publications

(124 citation statements)

References 20 publications

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“…11,12 WGH represent the second largest group of women with inherited blood clotting defects after patients with von Willebrand disease (VWD). 6,7,14 Despite the fact that the haemorrhagic phenotype of WGH with reduced clotting factor activity has been reported in literature to resemble that of mild haemophilia patients and that the recommended treatment is the same, 2 to our knowledge, this was the first study that directly compared WGMH and males with mild haemophilia. 6,7,14 Despite the fact that the haemorrhagic phenotype of WGH with reduced clotting factor activity has been reported in literature to resemble that of mild haemophilia patients and that the recommended treatment is the same, 2 to our knowledge, this was the first study that directly compared WGMH and males with mild haemophilia.…”

Section: Discussionmentioning

confidence: 95%

Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia?

et al. 2019

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Introduction Certain haemophilia carriers demonstrate an increased bleeding tendency, mainly related to clotting factor deficiency. No study has so far formally compared the bleeding phenotype of women and girls with mild FVIII or FIX deficiency and associated management with that of male patients affected by mild haemophilia A and B. Material and methods We retrospectively evaluated 44 women and girls with mild FVIII or FIX deficiency (FVIII or FIX 0.05‐0.5 IU/mL) and 77 male patients with mild haemophilia A or B and compared them with respect to clotting factor level, age at and trigger for diagnosis, as well as treatment modalities. Results After excluding gender‐related haemorrhagic symptoms, haemophilia carriers with plasma factor levels in the mild haemophilia range and male patients affected by mild haemophilia present a comparable haemorrhagic profile, mainly characterized by mucocutaneous and postinjury bleeding. Haemophilia carriers with clotting factor deficiency, however, distinguish themselves in terms of later age at diagnosis, higher mean factor levels and trigger for diagnosis. Conclusions Women and girls with mild FVIII or FIX deficiency should be considered as mild haemophilia patients and have access to care and management inspired from male haemophilia patients while integrating differences and specificities. Larger international studies comparing the clinical presentation and treatment modalities of mild clotting FVIII and FIX deficiencies in both haemophilia males and females should be encouraged.

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Section: Discussionmentioning

confidence: 95%

Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia?

et al. 2019

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“…4,13 Most subjects in our study were scored for menorrhagia based on their high frequency of sanitary napkin changes, at less than every 2 hours. Specific to China, the Chinese healthcare system is mostly hospital-centred and the delivery system is Heterogeneity between different cultural populations might also play a part in our observation that both our carriers and control females had a higher prevalence of menorrhagia than were observed in earlier reports.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4] A number of studies have clearly demonstrated increased bleeding tendency in haemophilia carriers compared to controls, with cutaneous bleeding, heavy menstrual bleeding and excessive/prolonged bleeding after invasive procedures and childbirth being the most common. [2][3][4] A number of studies have clearly demonstrated increased bleeding tendency in haemophilia carriers compared to controls, with cutaneous bleeding, heavy menstrual bleeding and excessive/prolonged bleeding after invasive procedures and childbirth being the most common.…”

Section: Introductionmentioning

confidence: 99%

“…central nervous system; GI, gastrointestinal; IUD, intrauterine device including (in descending order based on ISTH-BAT) menorrhagia, cutaneous bleeding, bleeding after surgery, oral cavity bleeding, bleeding after dental extraction and postpartum haemorrhage 3,4. Unique to our study is the inclusion of surgical abortion and IUD placement bleeding as subcategories to re-calculate the BS in the C-BAT.…”

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confidence: 99%

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

Fang²,

et al. 2019

Haemophilia

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Introduction: An increased bleeding tendency has been shown in female haemophilia carriers compared to healthy females. Bleeding assessment tools (BATs) have mainly been performed in western cultures. It is unclear how they perform in populations with different healthcare, health/wellness concepts and awareness, as well as family planning practices. Aim: To (a) describe and compare the bleeding symptoms in carriers with healthy females, particularly for bleeding after surgical abortion and intrauterine device (IUD) placement which are performed frequently for family planning in China; (b) quantify scores of International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) and Chinese-BAT (C-BAT) developed to include surgical abortion and IUD placement as separate categories in Chinese haemophilia carriers;(c) correlate bleeding scores (BS) with factor levels. Methods:We conducted a multicentre, cross-sectional study on obligate haemophilia carriers and healthy controls using ISTH-BAT and C-BAT. Results:We enrolled 125 haemophilia carriers and 106 controls. Carriers, compared to controls, had significantly higher median BS (3 vs 1 by both ISTH-BAT and C-BAT) and lower factor level (63.5 vs 101.8 IU/dL). Bleeding after surgical abortion and IUD placement was significantly associated with carrier status. Bleeding scores from neither ISTH-BAT nor C-BAT showed significant correlation with factor levels. Conclusion: Haemophilia carriers in China experienced abnormal bleeding. Uniqueto the Chinese carriers is significant bleeding after surgical abortion (3rd highest incidence of bleeding symptom) and IUD placement (4th highest). However, both ISTH-BAT and C-BAT exhibited no correlation between BS and factor levels in this population and neither could identify carriers with low factor level (of <50 IU/dL).

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“…This is promising in terms of efficiently generating useful data that reflects the bleeding severity experienced by these women, which should make the assessment of bleeding in haemophilia carriers more widely accessible. Interestingly, the sensitivity for the identification of a low FVIII/FIX level is higher for the Self‐BAT than for the ISTH‐BAT, where it was 57% in haemophilia carriers . The population studied with the ISTH‐BAT was more diverse; recruited from 7 centres throughout North American, Europe and South Africa, perhaps explaining the difference.…”

Section: Includes Information On All Haemophilia Carriers Enrolled (Nmentioning

confidence: 99%

Evaluation of the self‐administered bleeding assessment tool (Self‐BAT) in haemophilia carriers and correlations with quality of life

et al. 2017

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Evaluation of the utility of the ISTH‐BAT in haemophilia carriers: a multinational study

Cited by 72 publications

References 20 publications

Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia?

Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia?

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

Evaluation of the self‐administered bleeding assessment tool (Self‐BAT) in haemophilia carriers and correlations with quality of life

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