The Japanese Journal of Pediatric Hematology / Oncology
Online ISSN : 2189-5384
Print ISSN : 2187-011X
ISSN-L : 2187-011X
Original Article
Current status and problems of hemophilia carriers in a single institute
Yuka NanjoAtsushi SatoHiroe HayasakaHiromi WatanabeYuko NagoyaMaki OgawaTasuku SuzukiNobu SuzukiMasaei OnumaMasue Imaizumi
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2021 Volume 58 Issue 2 Pages 149-155

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Abstract

Hemophilia treatments are continuously improving; however, hemophilia carriers still face various problems. Specifically, the carriers’ bleeding risk is a pressing concern and a recent focus of attention. We conducted a survey on the presence or absence of a tendency to bleed and conditions at delivery in mothers of patients with hemophilia who were treated at our hospital. We interviewed 14 confirmed and 9 suspected hemophilia carriers (23 in total). We evaluated coagulation factor activity in 17 participants. Among the 17 participants, eight showed a factor VIII or IX activity level of lower than 40%. More than half of the confirmed carriers reported difficulty in achieving hemostasis after tooth extraction and hypermenorrhea. No clear correlation was found between bleeding tendencies (y/n) and coagulation factor activity. Eight of the 14 confirmed carriers self-reported excessive bleeding during and after childbirth; four experienced massive bleeding of 1000 mL or more at delivery and two received blood transfusion. In the two participants who underwent blood transfusion, the activity levels of coagulation factors during the nonpregnant period were 40% or higher. Both participants were not informed of the possibility of being a hemophilia carrier even with hemophilia patients in their family members. It has been revealed that there are more-than-expected females who are troubled with bleeding symptoms among hemophilia carriers. It is still considered that commonly these carriers have not received sufficient explanation from medical providers. Therefore, it is important for medical professionals that are involved in treating hemophilia to support these carriers by providing sufficient explanation and support to them.

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© 2021 The Japanese Society of Pediatric Hematology / Oncology
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