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A rare disorder characterized by sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding.
ORPHA:64743Classification level: Disorder
- Obliterative portal venopathy
- Prevalence: Unknown
- Inheritance: -
- Age of onset: All ages
- ICD-10: K74.1
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
Prevalence is unknown. Distribution is worldwide, with many cases reported in Asia. The disease may occur at any age. Both sexes are equally affected.
HPS typically manifests with overt splenomegaly with pancytopenia, portal hypertension and mild abnormalities in liver function tests. HPS patients are in good condition, but may present with ruptured esophagogastric varices. Most commonly, the condition is detected in investigating a fortuitous finding of hypersplenism or splenomegaly. Ascites, hepatic encephalopathy and jaundice are uncommon. Main histopathologic findings are periportal fibrosis, occlusion of small portal veins, sclerosis of the portal venous system, and proliferation of small vascular channels within/around portal tracts. The disease is slowly progressive. Liver failure, ascites or encephalopathy may occur in rare patients with an advanced stage of the disease. The risk of extrahepatic portal vein thrombosis is high.
The ætiology of HPS is unclear. Exposure to toxic substances (arsenic or vinyl chloride) or drugs (vitamin A, 6-mercaptopurine), autoimmune and connective tissue diseases, systemic or intraabdominal infections, and clotting abnormalities have been incriminated. A genetic background has been suggested.
HPS is usually a diagnosis of exclusion. A large liver biopsy specimen, together with expertise and awareness of the examiner, are requested for optimal diagnosis.
Differential diagnosis from liver cirrhosis is crucial and may be difficult.
Management and treatment
Treatment of active bleeding and its prophylaxis can follow the recommendations for patients with cirrhosis. In considering splenectomy, the high risk of thrombosis in the portal venous system should be taken into account.
Current overall prognosis is good (80% survival at ten years after the diagnosis). Male patients and patients with disease onset before 40 years of age show a poorer prognosis. Variceal bleeding is the main cause for morbidity and mortality.
- Review article
- English (2015)