Portal Hypertension in Patients with Hemophilia

Hemophilia is a congenital disorder that affects the blood's ability to clot properly. The condition predisposes patients to experience spontaneous bleeds, particularly into joints, or excessive bleeding following trauma or other insult.[1] Hemophilia is a hereditary x-linked recessive disorder that primarily occurs as one of two types. Type A occurs due to a deficiency in clotting factor VIII, while type B occurs due to a deficiency in clotting factor IX.[1] Hemophilia A and B are the most common of the severe hereditary hemorrhagic disorders.[1] There is a type C hemophilia, however, it is quite rare. Hemophilia afflicts roughly 400,000 people worldwide with hemophilia A accounting for greater than 80% of all hemophilia cases.[2] [3] Historically, patients with hemophilia have been treated with transfusions of clotting factor VIII or IX concentrates to correct their coagulopathy. The inability to pasteurize factor concentrates prior to the late 1980s led to many hemophiliacs contracting blood-borne viruses such as hepatitis B virus (HBV), hepatitis C virus (HCV), and human immunodeficiency virus (HIV).[4] As a result, there is an increased prevalence of viral hepatitis, chronic liver disease, cirrhosis, and, subsequently, portal hypertension in this patient population.[5]

Publication History

Article published online:
10 August 2023

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