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Clinical characteristics and prognostic factors of acquired haemophilia A in Korea

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Author(s)
Shin Young HyunHo-Jin ShinSung-Soo YoonJoon Ho MoonJae Joon HanDeok-Hwan YangWon Sik LeeSoo-Mee BangHo-Young YhimSung-Hyun KimDoyeun OhYoung Rok DoYong ParkChul Won ChoiJe-Hwan LeeJi Eun JangSoo-Jeong KimDoh Yu HwangJin Seok Kim
Keimyung Author(s)
Do, Young Rok
Department
Dept. of Internal Medicine (내과학)
Journal Title
Haemophilia
Issued Date
2021
Volume
27
Issue
5
Keyword
acquiredautoantibodiesfactor 8 deficiencyhaemorrhagehaemostasisimmunosuppressiveagents
Abstract
Introduction:
Acquired haemophilia A (AHA) treatment involves the haemostatic treatment for acute haemorrhage and immunosuppressive therapy (IST) to eradicate FVIII inhibitory antibodies.

Aim:
We assessed the clinical features of AHA and analysed treatment outcomes in Korea. We further identified prognostic factors affecting treatment outcomes.

Methods:
Medical records of 55 patients with AHA from 18 institutions were reviewed retrospectively. Logistic and Cox regression analyses were performed to elucidate clinical factors affecting the achievement of complete remission (CR). The primary endpoint was time to CR after IST, and secondary endpoints were time to haemostasis, the achievement of CR, and overall survival (OS).

Results:
Among the 55 patients, 50 (91%) had bleeding symptoms. Bleeding was severe in 74% of patients. Thirty-six (72%) patients received haemostatic therapy. Of the 42 patients who received IST, 23 (52%) received steroid alone, with a 52% response rate, and 10 (25%) received a combination of steroid and cyclophosphamide, with an 83% response rate. Five (16%) patients relapsed after a median duration of 220 days. There were eight deaths. In the Cox regression analysis, the FVIII inhibitor titre ≥ 20 BU/mL was the only significant prognostic factor affecting time to CR and haemostasis. No significant difference was observed in OS based on the inhibitor titre.

Conclusion:
The present study demonstrated the demographic data of AHA in Korea and showed that FVIII inhibitory antibody titre was a predictor of time to achieve CR after IST.
Keimyung Author(s)(Kor)
도영록
Publisher
School of Medicine (의과대학)
Citation
Shin Young Hyun et al. (2021). Clinical characteristics and prognostic factors of acquired haemophilia A in Korea. Haemophilia, 27(5), e609–e616. doi: 10.1111/hae.14370
Type
Article
ISSN
1365-2516
Source
https://onlinelibrary.wiley.com/doi/10.1111/hae.14370
DOI
10.1111/hae.14370
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/43798
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Internal Medicine (내과학)
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