Characteristics and outcome of a territory-wide cohort study of patients with acquired hemophilia A in Hong Kong

Sin, Chun-fung; Li, Ting Hon Stanford; Wong, Ka-ping; Wong, Ka-wai; Sin, Yuen-ting; Lam, Wing Kit; Mak, Hiu-chun; Lau, Wai-pun; Yeung, Ka-pik; Leung, Fung Shan Kate; Li, Chung-hin

doi:10.1016/j.thromres.2023.11.025

Cited by 3 publications

(1 citation statement)

References 23 publications

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“…AHA is a disorder that can be life threating for patients, with an estimated mortality rate of 7.9% to 22% [ 111 , 112 , 113 , 114 ]. AHA can be attributed to hematological malignancies, solid tumors, autoimmune disorders, pregnancy, drugs, and other factors, or it can be presented as an individual clinical disorder [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

Genetics and Epigenetics in Acquired Hemophilia A: From Bench to Bedside

Evangelidis,

Kotsiou,

Evangelidis

et al. 2024

CIMB

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Acquired hemophilia A (AHA) is a bleeding disorder characterized by the immunological inhibition of factor VIII (FVIII) of the hemostatic pathway leading to hemorrhagic events. Different domains of FVIII are the target of autoantibodies (mainly immunoglobulin (Ig) G) leading to the deficiency of FVIII. Several factors have been associated with the activation of the auto-immunity towards FVIII. Emerging evidence implicates CD4+ T cell activation in mediating this autoimmune response, with their involvement like that observed in congenital hemophilia A. Several genes such as HLA II DRB*16, DQB1*0502, and CTLA-4 + 49 are responsible for the pathogenesis of AHA. Epigenetic modifications and mainly long-coding RNAS (lncRNAs) are potentially contributing to the pathogenesis of AHA. The treatment approach of AHA includes the management of acute bleeding events and the administration of immunosuppressive medications. This review aimed to summarize the published data on the genetics and epigenetics of AHA. The severity and the mortality of this disease are creating an emerging need for further research in the field of the genetics and epigenetics of acquired hemorrhagic disorder.

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