Hepatoportal sclerosis (HPS) is a clinical disorder of obscure pathogenesis with a variable clinical profile. The aim of the study was to summarize the clinical features of Turkish patients with HPS and to measure the serum levels of interleukin (IL)-6 and interferon (IFN)-gamma to determine the T helper cell profile in the pathogenesis. The study was conducted on 34 HPS patients (17 men, 17 women; mean age at diagnosis, 27+/-10 years) and 15 healthy controls. The clinical features of HPS patients including demographics, clinical history, laboratory, and ultrasonography findings were summarized. Serum IL-6 and IFN-gamma levels were measured by using commercially available enzyme-linked immunosorbent assay kits. Gastrointestinal bleeding was the most common dominant presenting symptom. Majority of the patients had preserved liver function tests. Serum triglyceride levels were decreased in 30%. Abdominal ultrasonography revealed well-demarcated bands of increased echogenicity surrounding the portal vein wall and sudden narrowing of the intrahepatic second-degree portal vein branches in all cases. Spontaneous shunts and/or collaterals were seen in 13 cases (37%). Extrahepatic portal vein thrombosis were seen in 7 (20%) patients after at least 5 years of disease duration. Serum levels of both IL-6 (median, 3.2 pg/mL) and IFN-gamma (median, 7.8 pg/mL) were significantly higher in HPS patients compared with the control group (median, 1 pg/mL). HPS has variable clinical profile in different geographic areas of the world. Both Th1 and 2 cells may have a role in the regulation of immune response and pathogenesis of the disease.