Diagnosis, therapeutic advances, and key recommendations for the management of factor X deficiency
Makaleler > Derleme · 2021
Yazarlar: Flora Peyvandi, Guenter Auerswald, Steven K. Austin, Ri Liesner, Ramazan Kaan KAVAKLI, Maria Teresa Alvarez Roman, Carolyn M. Millar
BLOOD REVIEWS, SCI Expanded İndekslerine Giren Dergi, Uluslararası, 2021, cilt 50, ss 100833-100833
DOI: 10.1016/j.blre.2021.100833
Özet
Factor X deficiency is a rare coagulation disorder that can be hereditary or acquired. The typology and severity of the associated bleeding symptoms are highly heterogeneous, adding to the difficulties of diagnosis and man-agement. Evidence-based guidelines and reviews on factor X deficiency are generally limited to publications covering a range of rare bleeding disorders. Here we provide a comprehensive review of the literature on factor X deficiency, focusing on the hereditary form, and discuss the evolution in disease management and the evidence associated with available treatment options. Current recommendations advise clinicians to use single-factor replacement therapy for hereditary disease rather than multifactor therapies such as fresh frozen plasma, cry-oprecipitate, and prothrombin complex concentrates. Consensus in treatment guidelines is still urgently needed to ensure optimal management of patients with factor X deficiency across the spectrum of disease severity.