Risk factors, treatment and survival rates of late‐onset acquired haemophilia A: A cohort study from the Shizuoka Kokuho Database

Introduction Acquired haemophilia A (AHA) is a rare disease. The risk factors have yet to be studied. Aim We aimed to identify risk factors for late‐onset AHA in Japan. Methods A population‐based cohort study was conducted using data from the Shizuoka Kokuho Database. The study population was define...

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Published inHaemophilia : the official journal of the World Federation of Hemophilia Vol. 29; no. 3; pp. 799 - 808
Main Authors Shoji‐Asahina, Aya, Nakatani, Eiji, Imaichi, Yutaro, Ohata, Emi, Oshima, Michiko, Miyakoshi, Akinori, Miyake, Hiromu, Ichikawa, Yoshikazu, Dote, Hisashi, Ubukata, Nanako, Funaki, Daito, Urano, Tetsumei
Format Journal Article
LanguageEnglish
Published England Wiley Subscription Services, Inc 01.05.2023
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Summary:Introduction Acquired haemophilia A (AHA) is a rare disease. The risk factors have yet to be studied. Aim We aimed to identify risk factors for late‐onset AHA in Japan. Methods A population‐based cohort study was conducted using data from the Shizuoka Kokuho Database. The study population was defined as individuals aged ≥60 years. Cause‐specific Cox regression analysis was performed to calculate hazard ratios. Results Of 1,160,934 registrants, there were 34 patients with newly diagnosed AHA. The mean follow‐up period was 5.6 years, and the incidence of AHA was 5.21 per million person‐years. Myocardial infarction, diabetes mellitus, solid tumors, antimicrobial agents, phenytoin and anti‐dementia drugs, which showed significant differences in the univariate analysis, were excluded from the multivariable analysis because of the small number of cases. Multivariable regression analysis showed that the presence of Alzheimer's disease (hazard ratio [HR]:4.28, 95% confidence interval [CI]:1.67‐10.97) and rheumatic disease (HR:4.65, 95% CI:1.79‐12.12) increased the risk of AHA development. Conclusion We found that comorbid Alzheimer's disease is a risk factor of AHA incidence in the general population. Our findings provide insight into the etiology of AHA, and the proof of the coexistence of Alzheimer's disease may support the recent notion that Alzheimer disease is an autoimmune disease.
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ISSN:1351-8216
1365-2516
DOI:10.1111/hae.14793