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Background  Portal vein thrombosis (PVT) is a rare condition associated with serious morbidity and mortality. The objective of this study was to determine the frequency, clinical presentations, and risk factors of PVT from the set of data firstly collected among the Southeast Asian population.    Methods  A retrospective study was undertaken to identify patients diagnosed with thrombosis of the portal system and other abdominal veins. The hospital medical records were retrieved based on the selected ICD-10 codes. Clinical presentations were collected and risk factors determined.    Results  From 2000-2009, 467 hospital charts with designated ICD-10 codes of I81, I82.2, I82.3, I82.8, I82.9, or K55.0 were identified. PVT (I81) was the most common thrombosis (194 cases, 41.54%). The majority of PVT patients were males (65%), older than 40 years (75%), and presented with abdominal distension/ascites (69%), splenomegaly (54.6%), and abdominal pain (50.5%). Overall, the predominant risk factor was hepatocellular carcinoma (HCC) (52.5%), followed by liver cirrhosis without cancer (9.3%), abdominal infection/inflammation (9.3%), cholangiocarcinoma (8.2%), and abdominal intervention (7.7%). In young patients, abdominal interventions including umbilical catheterization (23.1%) and hepatectomy (7.7%) were the most frequent risks whereas in older cases, primary hepatobiliary cancer and cirrhosis (78%) were the major risks. Liver metastases from other organs were infrequently found. Chronic hepatitis B virus (HBV) infection was the main etiology associated with cirrhosis/HCC leading to PVT in this cohort. A third of the older PVT patients (age  > 40) had HBV and very few carried hepatitis C virus (HCV) whereas none of the young PVT patients (age <20) had HBV or HCV. A variety of abdominal infections/inflammations were also found including liver abscess, splenic abscess, cholangitis, cholecystitis, pancreatitis, omphalitis, and abdominal tuberculosis. Single cases of systemic lymphangiomatosis and Klippel-Trénaunay vascular malformation syndrome were also identified. Other thrombophilic conditions such as myeloproliferative neoplasms, paroxysmal nocturnal hemoglobinuria, protein S deficiency, and anti-phospholipid syndrome were rarely encountered.    Conclusion  HBV is the major risk of PVT in the Southeast Asian population. Several risk factors identified in this population have rarely been described and some are remarkably different from those reported in the West. Host and environmental factors may play a causal role in the initiation and development of PVT in various ethnicities and geographic locations.

High Incidence of Hepatitis B Infection-Associated Cirrhosis and Hepatocellular Carcinoma in the Southeast Asian Patients with Portal Vein Thrombosis