Progressive liver disease in haemophilia: an understated problem?

Area of Science:

• Hepatology
• Hematology
• Virology

Background:

• Patients with hemophilia receiving clotting factor concentrates are at risk for liver disease.
• Non-A, non-B hepatitis is a suspected cause of liver pathology in this population.

Purpose of the Study:

• To investigate the prevalence and progression of liver disease in patients with hemophilia.
• To identify the etiological factors contributing to liver disease in hemophilia patients.

Main Methods:

• An 8-year longitudinal study involving 79 unselected patients with hemophilia.
• Assessment of liver disease through clinical evaluation, laboratory tests (serum aminotransferases), and serial liver biopsies.
• Histological analysis to determine the cause of liver disease, including viral hepatitis.

Main Results:

• Evidence of chronic progressive liver disease in 17 (21%) patients.
• Diagnoses included chronic active hepatitis (8 patients) and cirrhosis (9 patients), with 5 showing esophageal varices.
• Histology implicated non-A, non-B hepatitis as the primary cause, though other viral influences were considered.
• Liver biopsies demonstrated progression from chronic persistent hepatitis to chronic active hepatitis and cirrhosis within 6 years.
• No correlation was found between serum aminotransferase levels and the severity of liver disease.
• Symptoms and abnormal physical signs were infrequent.

Conclusions:

• Chronic progressive liver disease is a significant concern in hemophilia patients treated with clotting factor concentrates.
• Non-A, non-B hepatitis is a major contributor to liver disease progression, which can be rapid.
• Chronic persistent hepatitis in hemophiliacs may not be benign and can progress to cirrhosis.
• Liver disease in hemophilia patients is likely an increasing clinical challenge.
• Routine monitoring of liver health is crucial for this patient population.