Natural History of Hepatitis C in HIV-negative Patients with Congenital Coagulation Disorders

BACKGROUND/AIMS:Knowledge of the natural history of hepatitis C is useful for counselling patients and planning treatment. More data are needed from unselected patient groups without concomitant disease. The aim of this study was to describe the natural history of hepatitis C, two decades after infection, in a homogeneous and well-defined group of HIV-negative patients with congenital coagulation defects who had not received specific therapy for chronic hepatitis C.METHODS:Medical history, physical examination, laboratory tests and abdominal ultrasonography were performed in 45 HCV-RNA positive, HIV-negative patients, mainly haemophiliacs, from a single centre. Patients were classified according to results of ultrasonography.RESULTS:Two patients had experienced an episode of variceal bleeding; all others were asymptomatic. None had ascites. HCV-RNA titres were >500000 copies/ml in 23 patients, genotype was 1 in 31 patients. Forty (89%) had elevated transaminases, liver synthesis function was diminished in 7 (16%), and platelet count in 8 (18%). Ultrasonography was normal in 26 (58%) patients, 12 (27%) had isolated splenomegaly, and 7 (16%) had liver nodularity compatible with cirrhosis. Univariate analysis disclosed higher transaminases and gammaGT, higher age at acquisition of infection and higher present age as risk factors for more advanced disease. Of these, only higher present age was an independent predictor in multivariate analysis.CONCLUSIONS:Median 19 years after infection, 58% of patients had no other signs of liver disease than raised transaminases, 16% had cirrhosis on ultrasonography. Only 2/45 patients had symptomatic disease. Higher present age is the main risk factor for advanced disease in this group.