Clinical spectrum and long-term outcomes of non-cirrhotic portal venous system thrombosis in Hispanic population

Abstract

Introduction and Objectives

Portal venous system thrombosis (PVT) outside the setting of cirrhosis is uncommon with limited information available about the etiological and clinical characteristics across varied racial and ethnic groups.

Materials and Methods

This retrospective cohort study examines the long-term outcomes of non-cirrhotic, Hispanic adults diagnosed with PVT at a single center in Mexico City between January 2000 and August 2023. Patients with conditions predisposing to PVT were excluded.

Results

We included 100 Hispanic adults diagnosed with non-cirrhotic PVT. Thrombophilia was identified in 49 %, with antiphospholipid syndrome (APS) being the most prevalent thrombophilia (23 %), followed by JAK2 mutation (18 %). Chronic PVT, observed in 70 % of cases, predominantly affected the portal vein (50 %), followed by porto-mesenteric (41 %) and porto-splenic (9 %) territories. At diagnosis, 55 % had esophageal varices. Anticoagulant therapy was administered to over half of the patients for >12 months. Over a median follow-up of 55 months, the 5-year risk of re-thrombosis was 24 %, and the 5-year risk of variceal bleeding (VB) was 45 %. The 4-year overall survival (OS) was 97 %. Comparative analysis between thrombophilia-associated and idiopathic PVT did not reveal significant differences in VB, re-thrombosis, and OS.

Conclusions

This study underscores the unique clinical profile of Hispanic patients with non-cirrhotic PVT, highlighting a high prevalence of APS and substantial risks of VB. These findings contribute to a better understanding of PVT in Hispanics and emphasize the importance of tailored management strategies. The generalizability of our results may be limited by the thrombophilia testing approach, the excluded populations, and its retrospective, single center nature.